Heme/onc

Question 1

Sickle cell kid is going away - what is she at risk for

A) typhoid fever
B. Hepatitis

Answer 1

A. Typhoid fever

Salmonella

Question 2

12 year old kid comes in with rib pain and a few weeks of fever. White count is normal. Chest X-ray shows mottled appearance of ninth rib with periosteal reaction and new bone formation. Most likely diagnosis?

A. Osteosarcoma
B. Ewing’s sarcoma
C. Osteoid osteoma
D. Osteomyelitis

Answer 2

B. Ewing’s sarcoma

• because of rib pain + fever = Ewing’s

Question 3

2 year old Asian boy presents with a history of URTI symptoms. He is pale. HR 130, grade III/VI systolic murmur. Stable. WBC 6 ? / Hgb 53 / plt 585. MCV 58, elevated RDW (no number). No retina given.

A. Transfuse RBCs
B. Start oral iron
C. Parenteral iron

Answer 3

A. Transfuse pRBCs

Hgb <70

Iron def vs thalassemia

Question 4

Hemangioma on left upper lip and jaw measuring 2-3cm. Other than regular surveillance of growth and development at regular appointments, what else to do ?

A. Optho
B. MRI
C. No additional testing
D. CBC in 6 months

Answer 4

C. No additional testing

Infantile hemangiomas

Question 5

14 y.o male with constitutional symptoms, weight loss, generalized lymphadenopathy, including a right supraclavicular node. Low cell counts. Splenomegaly present. EBV testing negative. What is your initial investigation ?

A. CXR
B. Abdominal CT
C. Bone marrow
D. TB skin test

Answer 5

A. CXR

Look for mediastinal mass

Question 6

10 year old girl with sickle cell disease and a history of stroke. Which treatment is most recommended given her history of stroke ?

A. Hydroxyurea
B. Transfusion with pRBCs
C. Folic acid

Answer 6

B. Transfusion with pRBCs

Question 7

Girl with pancytopenia, HSM, febrile and unwell looking. Ulcerated tonsils on exam. Most likely diagnosis ?

A. Acute lymphoblastic leukaemia
B. Lymphoma

Answer 7

A. Acute lymphoblastic leukemia

In cases of ALL the occurrence of a necrotic lesion on the inside of the cheek, a sore, enlarged or ulcerated tonsil, bleeding gums, acute stomatitis, epistaxis or rapidly developing cervical adenopathy is many times the first symptom of the disease.

Question 8

A 2y.o boy presents with pallor. He has been drinking 1L of milk a day, but eats a varied diet. Blood work is as follows: Hgb 49, MCV 80. RDW is 14%. Peripheral blood smear: normal. Hemoglobin electrophoresis: HbA and Hb S. Which of the following conditions is most likely ?

A. Transient erythroblastopenia of childhood
B. Iron deficiency anemia
C. Sickle cell disease
D. Congenital red cell aplastic

Answer 8

A. Transient erythroblastopenia of childhood

Normocytic anemia between 6 mo- 3year old

Is MCV 80 normal in 2 year old ?

Question 9

A newborn baby with platelets of 10. Mom also has low platelets. What is the diagnosis ?

A. Autoimmune thrombocytopenia
B. Alloimmune thrombocytopenia
C. TAR

Answer 9

A. Autoimmune thrombocytopenia
- mom has low platelets

For alloimmune thrombocytopenia (NAIT) = maternal platelets are normal

Question 10

A boy with Hemophilia A who is managed at home with recombinant factor VIII presents with increasing episodes of bleeding and hemarthrosis of the ankle despite medication compliance. Your next step:

A. Check FVIII and inhibitor levels
B. Add DDAVP to the current regime
C. Try another FVIII product

Answer 10

A. Check FVIII and inhibitor levels

Question 11

A 10 year old with sickle cell anemia presents with fever and respiratory distress. On CXR there is a new infiltrate. Your next most important step?

A. Order type and screen and transfuse
B. IV antibiotics
C. IV hydration at 2x maintenance
D. Start hydroxyurea

Answer 11

B. IV antibiotics = 3rd generation cephalosporin and macro life

Question 12

2 year old girl from Mediterranean background. What values are most representative of thalassemia minor ?

A. Hgb 100, MCV 75, RBC 2.61
B. Hgb 100, MCV 60, RBC 4.81
C. Hgb 80, MCV 75, RBC 2.81
D. Hgb 80, MCV 60, RBC 3.21

Answer 12

B. Hgb 100, MCV 60, RBC 4.81

Thalassemia minor = Hb not as low + high RBC + Mentzer index < 13

Patients with beta thalassemia trait almost always have:

• A hematocrit > 30%
• A mean corpuscular volume (MCV < 75fL)
• RDW tends to be normal (vs high in iron def)
• the total RBC cell count tends to be normal to increased = 4-5

Question 13

Kid with Burkitt’s and Tumor lysis syndrome. Urine pH 7.0. What’s next.

A. Hemodialysis
B. ACE inhibitor
C. Rasburicase

Answer 13

D. Rasburicase

Question 14

2 year old child with fever for the past 2-3 weeks (up to 39C), lymphadenoapthy and mild hepatosplenomegaly presents complaining of joint pain. There is no true arthritis, but complains of pain with movement of joints. Hgb 91, WBC 9 (45% lymph, 55% PMN), platelets of 110. What is your next step in establishing the diagnosis ?

A. BMA
B. Blood culture
C. ANCA, ESR and RF
D. EBV serologies

Answer 14

A. BMA

Controversial with D. EBV serologies

EBV usually does not have anemia or bony pain. I guess sick kids fellow said this was too acute and also only one cell line is low (platelets not very low). We do EBV first. If more decrease in counts or EBV negative, can do BMA.

Danielle’s notes
Bone marrow aspiration and biopsy indicated in work-up of pancytopenia and leukocytes is when:

1. Unexplained and significant depression of > 1 peripheral blood cell elements
2. Blasts on the peripheral smear
3. Leukoerythroblastic changes on peripheral smear
4. A/w unexplained lymphadenopathy or hepatosplenomegaly
5. A/w anterior mediastinal mass

Question 15

Child with hemihypertrophy. Other than Wilms tumors, what other neoplasm do you need to screen for ?

A. Neuro last Oma
B. Hepatoblastoma
C. No other neoplasms

Answer 15

B. Hepatoblastoma

Question 16

An 11 month old presents with a scaly rash all over, especially in the diaper area. He also has exopthalmos and HSM. Xrays show bony lucencies on the scalp. What is the likely diagnosis ?

A. Neuroblastoma
B. Langerhans cell histiocytosis
C. ALL

Answer 16

B. Langerhans cell histiocytosis

Question 17

A child has a distended abdomen. An ultrasound shows an adrenal mass and hepatomegaly. Which of the following tests will make the diagnosis?

A. MIBG scan
B. Serum AFP
C. Urine HVA/VMA
D. Abdo/chest CT

Answer 17

C. Urine HVA/VMA

Question 18

Baby with petechiae. PLT 12. After transfusion, PLT are 16. Mom’s CBC is normal. What is the best management ?

A. PLA1 negative platelets
B. IVIG

Answer 18

A. PLA1 negative platelets

Question 19

Newborn with trisomy 21. High WBC, thrombocytopenia, anemia, HSM, petechiae. What does he have ?

A. CMV
B. Sepsis
C. Transient myeloproliferstice disorder

Answer 19

C. Transient myeloproliferstive disorder

Occurs in 10% of T21

Question 20

A child with ALL finished chemo 1 month ago and is exposed to Varicella. How do you treat ?

A. VZV vaccine
B. VZIG
C. VZV vaccine and admit for IV acyclovir
D. Admit for IV acyclovir

Answer 20

B. VZIG

Live vaccines should be delayed until at least 3 months after the completion of chemotherapy or at least 24 months after BMT in the absence of graft- versus- host disease and a need for ongoing immunosuppressive

If the patient had varicella, should give IV acyclovir, but do not give it as prophylaxis

Question 21

Given this hemoglobin electrophoresis, what is the most likely diagnosis ?

Hgb A - none
Hgb A2- 2%
Hgb F - 75%
Hgb S - 25%

A. Sickle cell trait
B. Sickle cell disease
C. Beta thalassemia
D. Alpha thalassemia

Answer 21

B. Sickle cell disease - no Hgb A

In trait there is HgB A

This is sickle cell disease on hydroxyurea or sickle cell and beta thalassemia or could be a baby with sickle cell

HbF is > 70% at birth and this starts to decline by 3 months of age

Question 22

A man with hemophilia A married a women who is a carrier of hemophilia A. Assuming they have a daughter, what is the likelihood of having a child with hemophilia A?

A. 0%
B. 25%
C. 50%
D. 100%

Answer 22

C. 50%

X-linked recessive inheritance does dad passes on his only affected X chromosome to daughter (100%) and mother has a 50% chance of passing affected X chromosome on to their daughter. So they have a 50% chance of having a daughter affected with hemophilia A because you need 2 affected copies in a daughter ( or just one in a son).

Question 23

A young boy presents with a normochromic, normocytic anemia. He is stable. What is your next course of action?

A. Start iron supplementation
B. Refer to Heme for BMA
C. Follow up in 2 weeks

Answer 23

C. Follow up in two weeks

Likely TEC - an acquired benign red cell aplasia that occurs in previously healthy children < 4 years old

• normochromic and normocytic anemia
• associated with a severe reticulocytopenia
• platelet numbers are usually normal but neutropenia may occur
• underlying etiology is unknown
• self limiting (resolves within weeks to 2 months) and and does not recur
• frequently follows a viral illness although no single agent has been identified

Question 24

A child is getting large volumes of pRBC transfusion. What ECG complication do you expect to see ?

A. Peaked T- waves
B. U waves
C. Short PR interval

Answer 24

A. Peaked T waves

Large volumes of pRBCs can cause high potassium release (especially if it has been stored for a long time).

Earliest sign on ECG is peaked T waves. Then the P wave widens and flattens, PR segment lengthens, and P waves eventually disappear.

• prolonged QRS interva with bizarre QRS morphology
• High- grade AV block with slow junctional and ventricular escape rhythms
• Any kind of conduction block ( bundle branch blocks, fascicular blocks)
• sinus bradycardia or slow AF
• development of a sine wave appearance ( - pre-terminal rhythm)
• can have cardiac arrest from asystole, VFIB or PEA

U- waves = hypokalemia
Short PR interva = pre-excitation syndrome like WPW

Question 25

A baby is born precipitously at home to parents who both have type 3 von Willebrand disease. Which of the following would be the most appropriate treatment? A. Cryoprecipitate B.FFP C. Factor VIII/vWF concentrate D. dDAVP

Answer 25

C. Factor VIII/ vWF concentrate Von Willibrand is inherited via AD Type 3 = no production of vWF and low factor 8 Type 3 is AR and both parents have type 3 therefore all offspring would have type 3 since no normal allele to pass on

Question 26

``` A newborn baby is bleeding. How do you differentiate DIC from hemorrhagic disease of the newborn due to vitamin K deficiency ? A. Low platelets B. Low PTT C. High PT D. High fibrinogen ```

Answer 26

A. Low platelets - low in DIC; normal in vit k deficiency Other B. Low PTT - high in DIC because of consumption of factors, can be normal or high in vit K deficiency C. High PT = high in both vit K def and DIC D. High fibrinogen = usually low in DIC DIC = consumption of coagulation factors (2,5,8, fibrinogen) and platelets - can affect INR and PTT, and low fibrinogen Hemorrhagic Disease of the newborn = prolonged INR and PTT because vit k dependent factors are decreased (2, 7, 9, 10 = 1972)

Question 27

You are treating a 7 year old girl with ALL for a clot from her central line. Despite increasing doses of heparin you are not getting a therapeutic level. What is the cause for this ? A. Antithrombin III deficiency B. Protein C deficiency C. Factor V Leiden D. Factor VIII deficiency

Answer 27

A. Antithrombin III deficiency Insensitivity to heparin is seen in some individuals with AT deficiency. This occurs because heparins require AT to inactivate coagulation enzymes. In contrast, direct inhibitors of coagulation enzymes such as direct thrombin (factor IIa) inhibitors and direct factor Xa inhibitors do not require AT.

Question 28

Kid with 2.5 cm supraclavicular lymph node. 8kg weight loss in past 6 months. What do you do first ? A. IV Piperacillin B. iV fluids and allopurinol C. IV methylated D. Isoniazid

Answer 28

B. IV fluids and allopurinol Likely Hodgkin’s lymphoma with B symptoms. Patients commonly present with painless, non-tender, form, rubbery cervical or supraclavicular LAD and some degree of mediastinal involvement. Leukemia and lymphoma are at risk of TLS

Question 29

4 year old girl with fever, splenomegaly, diffuse lymphadenopathy, purpuric rash on legs. Ulcerated pharynx. WBC 24, Hgb 80, PLTs 20. Likely diagnosis ? A. Leukemia B. Lymphoma C. Mononucleosis

Answer 29

A. Leukemia

Question 30

7 year old child with anemia Hgb 40, BMA shows arrested erythroid precursors. Cause ? A. TEC

Answer 30

A. Transient erythroblastopenia of childhood

Question 31

Chemotherapy that causes low sodium and vomiting ? A. Vincristine B. Cyclophosphamide C. Anthracycline

Answer 31

B. Cyclophosphamide - alkylate guanine and inhibits DNA synthesis - s/e = n/v + myelosuppression + hemorrhagic cystitis + pulmonary fibrosis + sIADH + bladder cancer + anaphylaxis - mesna prevents hemorrhagic cystititis - Vincristine can also cause SIADH

Question 32

Sickler with dactylitis, Hgb 95, which will be a/w worse prognosis ? A. Anemia B. Dactylitis

Answer 32

B. Dactylitis

Question 33

Vincristine side effect ?

Answer 33

A. Peripheral neuropathy

Question 34

NAIT, what do you give ? A. PLA- 1 negative platelets B. IVIG C. Single donor platelets D. Steroids

Answer 34

A. pLA- 1 negative platelets

Question 35

4year old boy who presents with pallor after a viral illness. Hgb 88, reticulocytes 0. WBC and platelets normal. What is the most likely diagnosis ? A. TEC B. ALL C. Diamond- Blackfan anemia D. Iron deficiency anemia

Answer 35

A. TEC

Question 36

Baby with Hgb of 222, same Hgb at 4 weeks. What is this associated with ? A. Prematurity B. T21 C. The small twin in twin- to twin transfusion syndrome D. Cystic fibrosis

Answer 36

B. T21

Question 37

7 y.o boy presents with low ferritin and low Hgb. Other cell lines normal, exam normal. Has been on iron treatment for 3 months with good compliance. His ferritin and Hgb are still low. What is the next step? A. Anti- TTG B. Bone Marrow C. Upper GI D. Upper endoscopy

Answer 37

A. Anti- TTG Celiac

Question 38

A 14 year old boy presents with his third relapse of ALL. An experimental form of chemotherapy is proposed as a potential treatment. He refuses the treatment. Which is true ? A. He can refuse the treatment if he is deemed capable B. Treatment must proceed C. Parents should decide if treatment should proceed D. An ethics committee should decide if treatment should proceed

Answer 38

A. He can refuse the treatment if he is deemed capable

Question 39

Describes a kid with HUS; what do you do you see on smear ? A. Schistocytes B. Megakaryocytes C. Reticulocytes

Answer 39

A. Schistocytes

Question 40

What med can’t a G6PD kid take ? ``` A. Chloroquine B. Trimethoprim C. ASA D. Acetominophen E. Quinine ```

Answer 40

A. chloroquine Can’t take Sulpha drugs but trimethoprim alone should be fine unless combined with sulfa More common: sulfas, Sentra, niteofurantoin, chloramphenicol, primaquine, chloroquine, summer dimercaprol, moth balls (naphthalene), favs beans, infection, dapsone, anti-malarials, rasburicase, ASA

Question 41

Kid with what sounds like diamond blackfan anemia. What is most commonly associated ? A. Short thumb B. Dysmorphic facies

Answer 41

B. Dysmorphic facies - most common A. Short thumb - most specific

Question 42

Abnormal thumbs, anemia, no thrombocytopenia. Other findings? A. Absent radius B. Craniofacial abnormalities

Answer 42

B. Craniofacial abnormalities

Question 43

8 year old healthy child with 1.5 x 2 cm supraclavicular lymph node, firm, non-tender, mobile with no erythema. What to do ? A. Excisional biopsy B. PPD C. Bartonella serology D. EBV serology

Answer 43

A. Excisional biopsy

Question 44

Child with thrombocytopenia (platelets 15 x 109/L). Which finding would suggest a bone marrow ? A. Fever > 39.5 B. Previous steroid use C. HgB < 115 D. ANC < 1000

Answer 44

D. ANC < 1000

Question 45

2 month old baby had IVH bleed, diagnosed with Hemophilia A. What is best management/advice regarding immunizations? A. Postpone 2 month vaccination B. Give IM vaccine and put pressure x 10 min C. Give factor VIII before giving IM vaccine

Answer 45

B. Give IM vaccine and put pressure x 10 min Give vaccines on the day that factor 8 is given

Question 46

Child with head tilt, ataxia, nystagmus. Most likely diagnosis ? A. Posterior fossa hemorrhage B. Cerebellar astrocytoma C. Brainstem glioma D. Craniopharyngioma

Answer 46

B. Cerebellar astrocytoma

Question 47

Child (age?) with ataxia, diplopia and headaches. What is the most likely diagnosis ? A. Brainstem glioma B. Cerebellar astrocytoma C. Craniopharyngioma D. Ependymoma

Answer 47

B. Cerebellar astrocytoma Careful about the diplopia - considered a cranial nerve involvement which is usually more indicative of a brainstem glioma but usually should have more brainstem findings. The diplopia could be secondary to the 6th nerve palsy because of increased ICP Torticollis from cerebellar tonsillar herniation - then get head tilt.

Question 48

Picture given of large hemangioma over V1 to scalp and upper eyelid distribution with bluish tint. Baby presents with this facial lesion and high output CHF. Apart from thrombocytopenia what else would you expect to see on CBC or smear? A. Neutropenia and mild anemia B. Normal INR/PTT C. Schistocytes D. High fibrinogen

Answer 48

C. Schistocytes B- INR/PTT normal - would be prolonged D. High fibrinogen - would be low Kasabach Merritt Syndrome

Question 49

Wilm’s Tumor is most associated with ? A. Tuberous Sclerosis B. Fragile X C. Angelman’s syndrome D. Beckwith- Wiedeman

Answer 49

D. Beckwith- Wiedeman

Question 50

Sickle cell anemia most common neurological sequelae A. Silent stroke B. Clinical stroke C. Seizures

Answer 50

A. Silent stroke

Question 51

Teenage girl with SSD. Acute fever, jaundice, abdo pain. Suspect cholecystitis, confirmed with U/S. After treating for one week, what do you suggest ? A. Cholecystectomy B. Ursodiol C. ERCP

Answer 51

A. Cholescystectomy

Question 52

13 y.o, obese with HbSS and RUQ pain, tenderness, fever, guarding and jaundice. U/S shows multiple gallstones, dilated CBD, inflamed walls. You diagnose acute cholangitis and admit for hydration and triple antibiotics. After the acute process is treated, what do you suggest ? A. Transfusion therapy B. Ursodiol C. Cholecystectomy D. Nothing

Answer 52

C. Cholescystectomy

Question 53

A 9 month old ex 32 weeker takes 40oz of homo milk per day. Hgb is 60, MCV 50. She is treated with Fe 4 mg/kg/day for one month. On repeat testing her HgB is 62, MCV is 50, and her retics are 0.01. What to do next ? A. BMA B. Verify compliance C. Hb electrophoresis D. Jejunal biopsy

Answer 53

B. Verify compliance

Question 54

Which of the following would most likely be seen with iron deficiency? A. PICA

Answer 54

A. PICA Children with PICA are at increased risk for lead poisening, iron deficiency anemia, mechanical bowel problems, intestinal obstruction, intestinal perforations, dental injury and parasitic infections Treatment - combined behavioural, social and medical approaches are generally indicated

Question 55

15 year old girl with period for a couple of years. Epistaxis and menorrhagia. What to check for ? A. Von Willibrand’s B. Hemophilia c C. Factor V Leiden mutation

Answer 55

A. Von Willibrand’s The American College of obstetrics an Gynecology recommend that women who have menorrhagia be evaluated for non Willibrand disease. Platelet function disorders and other coagulopathies are also frequent causes of menorrhagia Adolescent girls presenting with menorrhagia as their primary bleeding manifestation may have bleeding controlled with OCP and DDAVP

Question 56

Which cancer will most likely have bone marrow infiltration ? A. Wilms B. Neuroblastoma C. Hepatoblastoma D. Bone cancer of some sort - (I think osteosarcoma)

Answer 56

B. Neuroblastoma - blue cells - Metastasis occurs via local invasion or distant hematogenlus or lymphatic routes. - regional or distant LNs, long bones, and skull, bone marrow, liver and skin - Need at least two bone marrow aspirates and biopsies Wilms - Mets within abdomen, lung, thrombus into IVC +/- right atrium Hepatoblastoma - regional LNs and lungs Osteosarcoma - Mets to other bones, lung

Question 57

18 month old male (13 kg) with history of excessive milk intake presents on routine physical exam with pallor. Subsequent bloodwork reveals Hgb 45, MCV 56. What would be the best management ? A. Limit milk to 500cc/day B. Start elemental iron 60mg PO TID C. Give pRBC transfusion 130cc D. Change to protein hydrolysate formula Or Hgb 48 in an 11 month old male with tachycardia, pallor, excess milk intake. A. Restrict milk intake to <500cc/day B. Fe TID at 15 mg/kg C. Transfuse 10cc/kg blood D. Change to protein hydrolysate formula

Answer 57

Answer A, A Would also start iron but dosing wrong for above questions No need to change formula Why not transfusion ? Hgb < 70 Peds in review Erythrocyte transfusion should be considered only if the anemia is causing severe cardiovascular compromise,- hypervolemia and cardiac dilation may result from rapid correction of the anemia

Question 58

An otherwise healthy girl present with pallor. Her iron, and ferritin levels are normal. Her bloodwork shows a HgB of 70 with an MCV of 50. What study is most likely to give you her diagnosis ? A. Bone marrow aspirate B. Hemoglobin electrophoresis

Answer 58

B. Hemoglobin electrophoresis Alpha thal will have normal Hb electrophoresis Beta thal will have higher HbA2

Question 59

What is the advantage of using leukoreduced white cells? A. Decreased hemolytic reactions B. Decreases infectious complications C. Decreases febrile transfusion reactions

Answer 59

B. Decreases infectious complications Exam tip: ``` Leukoreduction = reduces CMV Irradiation = prevents GVHD ``` All blood is leukoreduced. CMV negative is an additional step only needed in BMT patients. Irradiate blood = for BMT patients, SCID, neonates

Question 60

A 7 year old boy who has had recent personality changes, decline in school performance and visual changes. Which is the first diagnosis to rule out ? A. Brain tumor B. DM C. ADHD D. Depression

Answer 60

A. Brain tumor

Question 61

What is a risk factor for childhood leukemia ? A. Parent treated for Leukemia B. In utero radiation C. Maternal alcohol D. NF type 1

Answer 61

D. NF type 1 The possible risks from fetal or childhood exposure to lower levels of radiation such as from X-ray tests or CT scans, are not known for sure

Question 62

16 year old boy undergoing treatment for non- Hodgkin’s lymphoma. Forty- eight hours after his last chemotherapy, he develops mild dysuria and hematuria. Platelet count was 90 pre- treatment. He is happy and well looking. He is sexually active. What is the most likely cause of his hematuria? A. Cyclophosphamide induced hemorrhagic cystitis B. Thrombocytopenia due to myelosuppression C. Chlamydial urethritis D. Urine infection

Answer 62

A. Cyclophosphamide induced hemorrhagic cystitis

Question 63

You are seeing a 1 week old Chinese boy with 1 day of jaundice. His bilirubin is 270 (mostly indirect), Hg 95 retics 9% , mom is AB + and he is B +. He otherwise looks well. What is the diagnosis? A. Sepsis B. Thalassemia C. G6PD deficiency D. ABO incompatibility

Answer 63

C. G6PD def A- sepsis - baby looks well B- thalassemia- does not usually cause neonatal jaundice D- not ABO - mom is AB+

Question 64

Shwachman diamond baby. Which vitamin level would be normal ? A. Vit A B. Vit E C. Vit B12 D. Vit D

Answer 64

C. Vit B12 Think about pancreatic insufficiency - ADEK

Question 65

3 year old with sickle cell, with fever, cough, tachypnea, unwell. What do you need to rule out 1st ? A. Acute chest crisis B. PE C. Pneumonia D. Asthma

Answer 65

A. Acute chest crisis

Question 66

Sickle cell with recurrent chest crisis. What is he at increased risk of? A. Recurrent cerebral infarction B. Asthma C. Gallstones

Answer 66

A. Recurrent cerebral infarction Recurrent chest crisis leads to interstitial lung disease and pulmonary hypertension (not asthma). Asthma worsens acute chest crisis Stroke has been a/w proximate (within) 2 weeks or concurrent ACS, perhaps reflecting the impact of inflammation on cerebral blood flow and impaired oxygenation in a population with a high prevalence of cerebral vasculopathy.

Question 67

Child with aniridia, what investigation will this child need regularly? A. CBC B. Abdo U/S C. Echocardiogram

Answer 67

B. Abdominal U/S Aniridia means an absence of the iris or coloured part of the eye. WAGR W-wilm’s Tumor A- aniridia GU abnormalities Retardation

Question 68

What is the following is the most associated with mortality in a child with thalassemia major ? A. Fe overload B. Cardiomyopathy C. Hyperglycemia What is the number one cause of complications in beta thalassemia major ? A. Iron overload B. Megaloblastic anemia C. Cardiomyopathy D. Poor hematopoisis

Answer 68

A. Fe overload Iron overload leads to cardiomyopathy

Question 69

You are counseling a mother of a SCD patient who will be requiring blood transfusions. Which of the following would she be most likely to receive in a blood transfusion? A. Hep B B. Hep C C. Parvovirus B19 D. Variant of Creutzfeldt- Jacob disease

Answer 69

C. Parvovirus B19 Parvovirus B19 1:5000-20,000

Question 70

How do you calculate Mentzer index ?

Answer 70

Mentzer Index = MCV/RBC Mentzer index < 13 = thalassemia Mentzer index > 13 = IDA

Question 71

What picture is most consistent with thalassemia minor ? A. Hb 100, MCV 80, RBC 2.81 B. Hb 100, MCV 60, RBC 4.81 C. Hb 60, MCV 80, RBC 4.81 D. Hb 60, MCV 60, RBC 3.71

Answer 71

B. Hb 100, MCV 60, RBC 4.81 Mentzer index = MCV/RBC Mentzer index < 13 = thalassemia Mentzer index > 13 = IDA A. 80/2.81 = 28 B. 60/4.81 = 12 C. 80/4.81 = 16 D. 60/3.71 = 16

Question 72

A 3y.o Chinese boy sees you in the emergency room after falling off his bicycle 24h ago. On exam, you see a swollen right knee and a yellow bruise on the anterior chest. What would explain this ? A. Chinese herbal remedies B. Hemophilia C. Child abuse D. Von Willibrand disease

Answer 72

B. Hemophilia - hemarthrosis In vWD more mucosal involvement. Hemophilia in all ethnic groups Easy bruising and excessive mucosal bleeding may be the first signs of hemophilia BUT severe soft tissue bleeding and hemarthroses are the classic bleeding symptoms. VWD has three types and severe forms can have hemarthroses.

Question 73

When you order blood for preterm babies, why is it irradiated ? A. To decrease the risk of CMV transmission B. To prevent Graft vs Host Disease C. To prevent hemolysis D. To sterilize the blood

Answer 73

B. To prevent GVHD CPS statement = “Most Centers now y- irradiate blood to deactivate lymphocytes and prevent GVHD, which is rare in newborn infants” Risk of CMV is reduced by universal leukoreduction

Question 74

Child with ITP and no other concerning risk factors on exam or history. What will his platelet count be in one year from now ? A. Probably will be normal B. Depends on results from of the current bone marrow C. Depends on the treatment given

Answer 74

A. Probably will be normal 70-80% with acute ITP, which resolves within 6 months

Question 75

18 month old found to be pale on normal routine exam. Blood work shows Hb 80 (normocytic and normochromic), retics low, normal WBC and platelets. What additional test would you do ? A. Ferritin B. Coombs C. Bone marrow D. Hb electrophoresis

Answer 75

C. Bone marrow A. Ferritin = usually low in iron def anemia which is microcytic B. Coombs = retics would be high in hemolytic anemia D. Hb electrophoresis = (thalassemia has a microcytic anemia)

Question 76

A child receives a blood transfusion. She becomes febrile and develops chills. What is your management ? A. Continue with transfusion, give methylprednisone B. Stop transfusion, give steroids C. Stop transfusion, run IV TKVO D. Continue transfusion and slower rate

Answer 76

C. Stop transfusion, run TKVO

Question 77

Beckwith- Wiedeman syndrome U/S. A question about how frequently ultrasound should be done ?

Answer 77

1. Abdominal U/S examination q3 months until age 8 years to r/o Wilms tumor 2. Measure serum AFP concentration every 2-3months in the first 4 years of life for early detection of hepatoblastoma

Question 78

Which of the following is present in Tumor lysis syndrome ? A. Hyponatremia B. Hypokalemia C. Hyperuricemia D. Hyperphosphatemia

Answer 78

C. Hyperuricemia

Question 79

6y.o Chinese boy presents with multiple bruises of various ages after falling off his bike. What is the most likely diagnosis ? A. ITP B. Child abuse C. Hemophilia

Answer 79

Either A. ITP or C. Hemophilia

Question 80

An 8 month old baby presents with an abdominal mass, thrombocytopenia, systolic murmur and a murmur over the liver. Ultrasound shows a 3 cm mass in the liver. What would you do ? A. DIC work-up B. Bone marrow C. Pulmonary imaging D. Liver function tests

Answer 80

A. DIC work-up Kasabach- Merritt syndrome

Question 81

7 year old Jehovah’s Witness is involved in MVA. Bleeding and has hemoglobin of 50 and is unstable. A. Treat immediately B. Try to talk to the parents to consent for blood C. Wait for a court order to be able to give the blood

Answer 81

A. Treat immediately It unstable you are allowed to proceed

Question 82

You saw a 15 year old M with respiratory distress and bilateral wheeze in the ER. This was the first episode of wheeze. Resolved with IV methylprednisolone and ventolin in ER and he was sent home. One week later the radiologist is reviewing the film and notices a widened mediastinum. What is the most likely cause ? A. Hodgkin’s lymphoma B. Acute lymphocytic leukemia C. Thymoma D. Sarcoidosis

Answer 82

A. Hodgkin’s lymphoma Anterior mediastinal mass 1. HL/NHL, thymus, teratoma, thyroid

Question 83

Teenage sickler girl, sexually active has had previous transfusions. She presents looking pale and icteric, with abdominal pain. On exam her right upper quadrant is tender with no guarding. Her hemoglobin is 70, AST 60 and ALT 55. Her bilirubins are elevated. What is her diagnosis? ``` A. Liver vaso-occlusive crisis B. Acute cholecystitis C. Hemochromatosis D. Hep C E. Fits- High Curtis F. Viral hepatitis ```

Answer 83

Controversial question A vs B (liver Vaso-occlusive crisis vs acute cholescystectomy) And what about Fitz High Curtis :(. A- hepatic sickle cell crisis occurs in 10% of patients with RUQ pain, fever, leukocytes is, jaundice. Generally self-limited and symptoms resolve in 1-3weeks B- could be possible but if it’s just cholecystitis your bili shouldn’t be high unless there is a blockage

Question 84

Teenager (or 7y.o) with sickle cell disease presents with cough and fever. CXR shows pneumonia. She looks toxic, fully immunized. What do you treat her with ? A. Cefuroxime and erythromycin B. Ceftriaxone and erythromycin 7 y.o with sickle cell and pneumonia, fully immunized. What do you treat her with ? A. Cefuroxime and biaxin B. Ceftriaxone and biaxin

Answer 84

B. Ceftriaxone and erythromycin General cephalosporin and macrolide Biaxin = clarithromycin

Question 85

Best prognostic sign in neuroblastoma? A. Age under 1y.o B. Unilateral disease

Answer 85

A. Age under 1 y.o ``` Young age is better < 12-18 months Other prognostic factors -stage -histology -N-Myc amplification (low risk if not amplified ) - ploidy (hyperploidy confers better prognosis) - 1p deletion (worse outcome) -17q gain (worse outcome) ``` Fun facts: neuroblastoma is a/w these things: - hirschsprung - central hypoventilation syndrome - NF1 - Beckwith - Wiedeman

Question 86

12 year old M with recent change in behaviour, irritability, daily headaches and a change in his vision. What is the most important thing to consider ? A. Brain tumor B. Drug use C. Psychiatric diagnosis

Answer 86

A. Brain tumor

Question 87

16 year old girl with SCD presents with cough and fever. CXR reveals LLL pneumonia. She looks toxic. How do you treat ? A. Cefuroxime and erythromycin B. Amoxicillin C. Erythromycin D. Ceftriaxone

Answer 87

D. Ceftriaxone. Ideal answer would be ceftriaxone and macrolide Cefuroxime = second generation. Should use a third generation cephalosporin like cefutaxime or ceftriaxone

Question 88

Child with SCD. Least likely invader? A. Pneumococcus B. Meningococcus C. HIB D. Staph aureus Child with SCD. What is he NOT at risk for? A. H. Flu B. S. Pneumonia C. N. Meningitides D. S epidermidis

Answer 88

D. Staph Really would be at risk for all but more at risk for encapsulated bacteria

Question 89

What is NOT necessary to ask the mother of A 9 month old presenting with anemia ? A. Duration of pregnancy B. Folate and B12 consumption during pregnancy C. Perinatal asphyxia D. History of delayed cord clamping or perinatal hemorrhage

Answer 89

C. Perinatal asphyxia A. Duration of pregnancy- preterm babies are at risk for anemia B. Maternal B12 deficiency is a risk factor for baby having vit B12 def D. Delayed cord clamping helps to prevent anemia in the neonatal period

Question 90

7 y.o boy, previously well. Post tonsillectomy bleeding. Increased PTT, normal platelets, normal factor levels. What do you do ? A. Amicar B. DDAVP C.Factor VIII D. Cryoprecipitate

Answer 90

D. Cryoprecipitate Controversial Answer: hhmmm I feel like I would say A because Nelson’s says to use it for oral surgeries. If factor 8 and vWF I would choose that. This could be hemophilia or vWD Apparently the TO lecturer said it could be A, B or D. Some sources say to avoid cryo if possible as it is a blood product so if you can avoid it try something else Medscape says: for prophylaxis in major surgery or for treatment of serious bleeding episodes, rVWF (with or without FVIII) or vWF- containing FVIII concentrates are the treatment of choice. ``` VWD Prolonged PTT and anemia Type 1: DDAVP Type 2,3: use VWF concentrate For al types of vWD, adjunct therapy should be considered when possible, such as the use of antifibrinolytics for oral surgery or hormonal tx for menorrhagia. ``` Amicar - aminocaproic acid is an antifibrinolytic and is a good treatment for mucosal bleeding, but I think this is considered an adjunct treatment. Cryoprecipitate- contains fibrinogen, factor 8, factor 13, vWF.

Question 91

3 y.o with pallor, leg pain and fatigue X 3 weeks. Exam reveals tired, pale anicteric child with HSM. No effusion or warmth/erythema of the legs. Most likely to confirm the diagnosis ? A. BMA B. U/S

Answer 91

A. BMA (or CBC and smear to look for blasts Presenting signs and symptoms of leukemia reflect bone marrow infiltration and extent of extramedullary disease spread - 1. Low Hgb: pallor, fatigue, irritability 2. Low white cells: fever, sepsis 3. Low platelets: bruising/bleeding, petechiae 4. Leukemic blasts: bone pain, limp 5. Extramedullary disease: LAD, HSM, gingival hyperplasia, leukemia cutis, chloromas, lymphadematous mass, CNS disease, testicular disease

Question 92

Poor prognosis with histiocytosis: A. Pancytopenia B. Lymphadenopathy C. Chronic lung disease D. Vertebral body involvement

Answer 92

A. Pancytopenia Langerhans cell histiocytosis

Question 93

4y.o has a chest X-ray done for a different reason, but it shows an asymptomatic solid circular lesion in the anterior mediastinum: ``` A. Ganglioneuroma B. Neuroblastoma C. Lymphoma D. Teratoma E. Mets from a Wilms Tumor ```

Answer 93

D. Teratoma As he is asymptomatic and was found incidentally. DDX of Anterior Mediastinal mass: 4 Ts 1. Thymus 2. Terrible lymphoma 3. Teratoma 4. Thyroid

Question 94

A screening CXR done on a 4year old reveals a posterior mediastinal mass. Dx ? A. Lymphoma B. Teratoma C. Neuroblastoma D. TB

Answer 94

C. Neuroblastoma

Question 95

A 9 y.o boy presents to the ER department with unexplained bruising for the past 24h. There is a history of previous bruising or excessive bleeding. The patient has been very healthy during the past year. The patient is looking well and shows no signs of acute distress. A complete CBC has the following findings: Hgb 115, WBC 10 500 and platelet count is 45000. Large platelets are seen on the smear. Treatment: A. IV immunoglobulin B. No specific treatment is needed at this time C. Oral prednisone D. Platelet transfusion

Answer 95

B. No specific treatment is needed at this time ?ITP ? I assume they meant no previous history of bruising?. The options are clearly related to ITP.

Question 96

A 6y.o girl has diffuse aching in her arms, legs and back for more than 2 weeks. Results of laboratory tests include hemoglobin 94g/dl, WBC 5,600 with no abnormal cells noted on smear and platelet count 106, 000. Radiographs of bone reveal osteopathic lesions and radio Luce this metaphyseal growth arrest lines. Of the following the most likely cause of these findings: A. ALL B. Aplastic Anemia C. Gaucher disease D. Multi focal osteomyelitis

Answer 96

A. ALL X-ray findings in leukemia - transverse radiolucent metaphyseal growth arrest lines - periosteal elevation with reactive sub periosteal cortical thickening - osteolytic lesions - diffuse osteopenia/osteoporosis

Question 97

A 3y.o child has a somewhat unsteady gait, regression of language and is irritable. PE: mild left facial weakness, brisk reflexes, +ve babinski and mild hypertonicity of the left upper and lower extremities. Most likely diagnosis: A. Cerebellar astrocytoma B. Pontine glioma C. Post infectious encephalitis D. Tumor of the right cerebral hemiphere

Answer 97

B. Pontine glioma Localized to brainstem over cerebellum because in addition to ataxia/language difficulties, also has UMN signs and ?CN involvement (left facial weakness). UTD article called diffuse pontine glioma: - clinical presentation: CN palsies, long tract signs (ie: hemiparesis) and ataxia - CNs VI and VII are most commonly affected but III, IV, IX and X may also be involved - hydrocephalus with elevated ICP observed in <10% at presentation

Question 98

Which of the following is most likely a sign of neutropenia ? A. Disseminated herpes zoster B. Mucosal ulceration C. Subcutaneous nodules D. Urticaria

Answer 98

B. Mucosal ulceration

Question 99

All are features of iron deficiency anemia EXCEPT: ``` A. Pica B. Koilonychia C. Cheilosis D. Mild scleral icterus E. Psychomotor retardation ```

Answer 99

D. Mild scleral icterus Apparently can cause a blue sclera ! Up to date - fatigue - weakness - headache - irritability - exercise intolerance - exertional dyspnea - vertigo - angina pectoris - pallor - dry or rough skin - blue sclera - atrophic glossitis with loss of tongue papillae, which may be accompanied by tongue pain or dry mouth - cheilosis (also called angular cheilitis) - koilonychia (spoon nails) - esophageal web, which may be accompanied by dysphagia ie; Plummer-Vinson or Patterson-Kelly syndrome; rare) - Alopecia (rare ) in especially severe cases - chlorosis (pale, faintly green complexion; extremely rare )

Question 100

What is the most common cause of febrile reactions in blood transfusions? A. Sensitization to WBC antigens B. Hemolysis C. Can’t remember the other choices

Answer 100

A. Sensitization to WBC antigens Risk of febrile reaction = 1/300, risk of hemolytic reaction = 1/6000

Question 101

In ITP: A. You will find a big spleen B. No proven link to preceding viral infection C. CNS intracranial bleed is the worse complication

Answer 101

C. CNS intracranial bleed is the worse complication

Question 102

A 3 year old girl with fever, arthralgia and lethargy for 10 days has lymphadenopathy, moderate hepatosplenomegaly, no obvious arthritis but screams in pain with minimal examination. WBC 9.5, Hgb 98, PLTs 140, smear normal. Next test: A. Bone marrow aspirate B. EBV titers C. Follow

Answer 102

Answer: A - they want to make sure you know this could be leukemia Hmmm I feel like the next test = EBV. If CBC and smear was there that is what I would pick. BMA will give you the diagnosis. Definitely NOT C.

Question 103

6 y.o girl with head tilt, diplopia, bilateral papilledema, and ataxia A. Craniopharyngioma B. Dandy Walker cyst C. Brainstem glioma D. Cerebellar astrocytoma

Answer 103

D. Cerebellar astrocytoma

Question 104

An 8 year old girl presents with fatigue. Blood work shows: hemoglobin 80, MCV 50, ferritin 150mcg/L, serum iron 50mmol/L. What is your next test ? ``` A. Barium meal B. Osmotic fragility test - spherocytosis C. Abdominal Tc 99 scintigraphy D. Hemoglobin electrophoresis E. Bone marrow biopsy ```

Answer 104

D. Hemoglobin electrophoresis We are thinking thalassemia Microcytic anemia with normal ferritin We don’t have any information as to whether this is a hemolytic anemia or not. T99 scan = look for active bleeding. Would usually have a normocytic anemia with active bleeding BMA - don’t know if her other cell lines are affected but likely not next step.

Question 105

A 2 year old is in your office for a routine check-up. His mother had no complaints. Growing well. You note pallor and do some blood work. Hemoglobin 80, low retic count. Smear is normocytic normochromic. Next investigation: A. Bone marrow aspirate B. Hemoglobin electrophoresis C. Coomb’s test D. Serum ferritin

Answer 105

Answer A - BMA - seems to be jumping the gun but the other tests would be looking at causes of microcytic anemia. And retics would be high if this was a hemolytic anemia. For Coombs. This can be done by process of elimination: - Hgb electrophoresis is looking for thalassemia (microcytic) or HbSS (would have high retics) - Coombs test - is looking for hemolytic anemia (should have high retics) - ferritin is looking for Fe deficiency (would be microcytic) Causes of normocytic anemia’s 1. Anemia of chronic disease/inflammation 2. RBC aplasia - TEC, infectious, drugs 3. Endocrinopathies 4. Renal failure 5. Acute bleeding 6. Hypersplenism 7. Congenital dyserythropoietic anemia II 8. Hemophagocytic syndrome (HLH)

Question 106

Characteristics of Scwachman- Diamond Syndrome A. Decreased pancreatic enzyme excretion B. Normal neutrophil count C. Hypocellular bone marrow D. Increased risk of diabetes mellitus

Answer 106

A. Decreased pancreatic enzyme excretion Shwachman-Diamond syndrome - AR disease- inherited pancytopenia Signs and symptoms of SDS include pancreatic insufficiency; neutropenia which may be cyclic, neutrophil chemotaxis defects, metaphyseal dysostosis, FTT, and short stature. Some patients with SDS have liver or kidney involvement, dental disease or learning difficulty. SDS is a common cause of congenital neutropenia. Skeletal abnormalities - advanced bone age, metaphyseal dysplasia, short or flared ribs, thoracic dystrophy, bifid thumbs. - thrombocytopenia in 70% - anemia in 50% Can turn into a myelodysplastic syndrome and transform into AML in 24%.

Question 107

Most consistent with DIC: ``` A. Increased fibrinogen B. Increased PTT C. Decreased PT D. Decreased INR E. Decreased fibrin split products ```

Answer 107

B. Increased PTT

Question 108

Better prognosis in neuroblastoma is associated with: ``` A. Female B. Age less than 1 year C. High excretion of VMA D. Normal blood pressure E. Unilateral ```

Answer 108

B. Age less than 1 year

Question 109

Paraneoplastic syndrome a/w neuroblastoma: ``` A. Chorea B. Athetosis C. Diarrhea D. Hypertension E. Hypercalcemia ```

Answer 109

C. Diarrhea Referring to Kerner- Morrison syndrome - intractable secretory diarrhea due to tumor secretion of Vasointestinal peptides

Question 110

A patient is being assessed for a bleeding diathesis. Labs show PT 12s, aPTT 50s, bleeding time 5 min, PLTs 250,000. (Normal range for bleeding time was listed on exam as < 5 min). PT 12s is normal (11-15 s). A. Hemophilia B. Von Willibrand’s disease C. Vitamin K deficiency D. Factor V deficiency

Answer 110

A. Hemophilia The question is unclear. It could be A (although should not have prolonged bleeding time) or it could be vWD (type 2). Answer A - if you consider the bleeding time as normal. Von Willibrand’s will have a prolonged bleeding time. Jeld was also confused - they said Jack said in the lecture the better answer is A and that bleeding time isn’t really done anymore.

Question 111

Poor prognosis in ALL is suggested by: ``` A. Female B. Age < 1 year C. CALLA positive D. Mediastinal mass E. Splenomegaly ```

Answer 111

B. Age < 1 year Poor prognostic factors for ALL 1. Age <1, >10 2. CNS disease 3. AML1 (bad), MLL (Bad) 4. BCR-ABL 5. Chromosomes <45 6. MRD end of induction - evidence of disease post 1 month induction therapy 7. WBC > 50,000 initially 8. TEL (good prognostic)

Question 112

A blood smear of a patient with iron deficiency anemia would show all EXCEPT: ``` A. Heinz bodies B. Poikilocytes C. Microcytosis D. Hypochromasia E. Normal or low reticulocyte count ```

Answer 112

A. Heinz bodies - seen in hemolytic anemia and G6PD - represents denatured Hb, also thalassemia

Question 113

In which of the following is platelet transfusion indicated ? ``` A. Aplastic anemia B. Acute ITP C. Chronic ITP D. HUS E. ITP secondary to Quinidine toxicity ```

Answer 113

A. Aplastic anemia

Question 114

A girl presents with Hb 80 and MCV 60. Ferritin and iron are normal. Next test: A. Hb electrophoresis B. Barium swallow C. Bone marrow

Answer 114

A. Hb electrophoresis

Question 115

Transient fever in blood transfusions is usually a result of: A. Sensitization to WBC antigens B. Acute GVHD C. Hepatitis C D. Hemolysis

Answer 115

A. Sensitization to WBC antigens

Question 116

What is the most common complication of hereditary spherocytosis: A. Aplastic anemia B. Gallstones C. Splenic infarcts D. Frontal bossing

Answer 116

B. Gallstones Pigmentary gallstones from as early as 4-5 years in 50% of unsplenectomized patients although they can be asymptomatic

Question 117

Opsoclonus is seen in which of the the following: ``` A. AML B. ALL C. Neuroblastoma D. Medulloblastoma E. Rhabdomyosarcoma ```

Answer 117

C. Neuroblastoma Myoclonoc jerking and random conjugate eye movements with or without cerebellar ataxia. Often a/w a biologically favourable and differentiated Tumor. The condition is likely immune mediated, may not resolve with tumor removal, and often exhibits progressive neuropsychological sequelae.

Question 118

A child with thalassemia major on recurrent transfusions and desfuroxime. What is the most serious complication: A. Cardiac hemisiderosis

Answer 118

A. Cardiac hemosiderosis

Question 119

Wilm’s Tumor is associated with all the following except: ``` A. 11p- B. NF C. Wiscott- Aldrich D. Aniridia E. Beckwith-Wiedeman ```

Answer 119

C. Wiscott- Aldrich Associated syndromes: 1. WAGR - wilm’s Tumor, aniridia, genitourinary abnormalities, mental retardation 2. Denys-Drash syndrome - early onset renal failure, male pseudohermaphrodism 3. Beckwith Wiedeman Best characterized Wilm’s Tumor gene is the gene WT, located at 11p13. In NF1, the incidence of Wilm’s Tumor is higher than in the general population.

Question 120

A VWD patient presents with bleeding. Which is the best treatment: A. FFP B. DDAVP C. Cryoprecipitate D. Platelets

Answer 120

C. Cryoprecipitate = factor 8 and fibrinogen but risk for viral transmission DDAVP - if not severe bleeding but will not work for all types of VWD types.

Question 121

Child with new ataxia. On exam irregular eye movements and some jerking of limbs. Most likely diagnosis ? 1. Brainstem glioma 2. Juvenile myoclonic epilepsy 3. Neuroblastoma 4. Acute cerebellar ataxia

Answer 121

3. Neuroblastoma

Question 122

Child described with cleft palate, TOF, low Calcium (features of DiGeorge). CBC shows Hb 110, PLT 100. Baby blood type AB+ (No mention of maternal PLT results). He is going for cardiac surgery. If he has blood loss what do you do ? A. Give him irradiated blood B. PLA1 platelets C. Maternal washed platelets D. O neg blood

Answer 122

A. Irradiated blood He is immune compromised.

Question 123

Girl with sickle cell who has had a stroke. How do prevent secondary stroke ? A. Hydroxyurea B. Folic acid C. Transfuse pRBCs

Answer 123

C. Transfuse pRBCs

Question 124

30 month old with easy bruising with bruises on bony prominences and thorax, otherwise exam unremarkable, thrombocytopenia 23 with large platelets on smear, no blasts, Hb 125, WBC 125, WBC 9.1. What is the MOST appropriate management ? A. 1g/kg IVIG once with close follow up B. 2 mg/kg prednisone x 4d with close follow up C. Avoid high risk activity and NSAIDs and close follow up

Answer 124

C. Avoid high risk activity and NSAIDs and close follow up This is ITP in the no or mild bleeding category

Question 125

Babe - 8 month old with neuroblastoma A. What are two investigations needed for work-up ? B. What is the stage this baby would be if the investigations were negative ?

Answer 125

A. Two investigations 1. Urinary or serum catecholamines 2. Bone marrow - to look for small round tumor cells 3. CT scans B. Stage 1 - localized tumor with complete gross excision +/- microscopic residual disease; representative ipsilateral LNs negative for tumor microscopically ( nodes attached to and removed with the tumor may be positive).

Question 126

3 year old immigrated from West Africa. Appears well, has scleral icterus. Otherwise normal exam. Given CBC - normocytic anemia. Hg electrophoresis HgF 50%, HgC 50%, HgA 0%. A. What is the disease? B. What are two complications this patient is at risk for?

Answer 126

A. What is the disease ? -HbCC B. What are two complications this patient is at risk for ? 1. Mild anemia 2. Splenomegaly 3. Cholelithiasis 4. Rare cases of splenic rupture have been reported Sickling does not occur. This condition is usually diagnosed through newborn screening. HbC crystallized disrupting the red cell membrane and HbC crystals may be visible on peripheral smear

Question 127

What are 3 indications to use oral propranolol for infantile hemangiomas ?

Answer 127

``` Infantile hemangiomas - oral propranolol Indications for use 1. Significant cosmetic issues 2. Life or vision- threatening 3. Ulcerated infantile hemangioma that is not responding to other treatment ```

Question 128

Two indications for exchange transfusion in a patient with sickle cell ? (Not sure of this was a separate question or part of sickle cell question)

Answer 128

Two indications for exchange transfusion in SCD 1. ACS 2. Stroke - used chronically to prevent stroke by keeping HgB S < 30% 3. Splenic sequestration 4. Priapism 5. Aplastic crisis 6. Pre-op

Question 129

A 3 year old girl presents with a 2 day history of acute onset head tilt. Other than papilledema, name 4 physical exam findings that would be consistent with an infratentorial Tumor ?

Answer 129

Infratentorial Tumor 4 PE findings 1. Truncal or gait ataxia, manifested by a broad- based gait or difficulty with heel- to toe walking (midline tumors) 2. Nystagmus 3. Dysmetria or finger-to- nose testing, intention tremor, and difficulty with heel- to -shin testing 4. Diplopia especially with lateral gaze (CN deficit)

Question 130

Name three symptoms of infratentorial brain tumor.

Answer 130

1. Symptoms from increased ICP = H/A, nausea/ vomiting 2. Clumsiness, worsening handwriting 3. Slow or halting speech 4. Double vision = diplopia

Question 131

Term newborn infant with petechial rash over the chest, but otherwise clinically very well. Physical exam is normal. CBC demonstrates platelets of 55. The pregnancy was normal and his mother is healthy. His mother’s CBC is normal. What are five possible causes of his low platelets ?

Answer 131

Low platelets - 5 causes 1. Neonatal alloimmune thrombocytopenia (NAIT) 2. Perinatal asphyxia 3. Drug induced: antiepileptic, digoxin in mother 4. Pre-eclampsia 5. TORCH infection (CMV) 6. Peripheral consumption: kasabach- Merritt phenomenon or hypersplenism Other causes: 1. Genetics = T21, TAR, WAS, congenital amegakaryocytic thrombocytopenia 2. Renal vein thrombosis 3. DIC and thrombosis die to sepsis, NEC (not well looking )

Question 132

Neonate with large tongue and hypoglycaemia. A. What is the diagnosis ? B. What is the pathophysiology of the hypoglycaemia ?

Answer 132

A. Beckwith- Wiedeman syndrome | B. Hyperplasia of the islet cells leads to hyperinsulinism.

Question 133

Anemia with Ferritin of 5. Determined to be nutritional. A. What are two nutritional pieces of advice to give ? B. What is the appropriate treatment ? C. How long should the treatment be provided ? D. When should the patient be brought back for follow-up ?

Answer 133

A. 2 nutritional pieces of advice to give ? 1. Limit cow’s milk consumption to no more than 16 oz (600ml) per day 2. Encourage at least 3 servings per day of iron containing foods (ie: fortified breakfast cereal, 3 oz of meat or 4 oz of tofu); children who eat less than this target usually have suboptimal iron intake and may benefit from an iron supplement. B. What is the appropriate treatment ? Iron supplementation 3-6mg/kg/day ie: ferrous sulfate C. How long should the treatment be provided for ? Until you can correct Hb and Ht + 2-3 months additional treatment for iron stores D. When should the patient be brought back for follow-up? If severe 1-2weeks after, if mild 4 weeks after * may need to update with new CPS statement

Question 134

Name five genetically inherited syndromes a/w leukemia.

Answer 134

1. Down syndrome 2. NF1 3. Bloom syndrome 4. Ataxia telangiectasia 5. Fanconi anemia 6. Li- Fraumeni syndrome (TP53) 7. Wiscott- Aldrich syndrome 8. Schwachman- diamond syndrome

Question 135

Young boy with iron deficiency anemia, confirmed on dietary history. A. How would you treat this (be specific)? B. How would you follow this ? C. What is the duration of therapy?

Answer 135

A. How do you treat ? - elemental iron 3-6mg/kg/day divided BID B. How would you follow this ? - repeat CBC and retic count in 1 month - expect Hb to rise by 10-20 in the first month C. What is the duration of therapy ? - treat for 2-3 months after correcting Hb level to replenish iron stores

Question 136

A 6 year old boy was recently diagnosed with ALL. A. List four biochemical markers seen in Tumor lysis syndrome B. List three management strategies for Tumor lysis syndrome

Answer 136

A. Four biochemical markers 1. Hyperkalemia 2. Hyper PO4 3. Hypocalcemia 4. Elevated urate 5. Elevated LDH 6. Metabolic acidosis B. Three management strategies 1. Hydration 1.5 maintenance and maintain good urine output 2. Frequent biochemical analysis Ie BID and more frequent depending on severity of initial Tumor lysis labs 3. Decrease urate levels - allopurinol/ rasburicase 4. Treat hyperkalemia + hyper phosphate is but do not treat hypocalcemia 5. Consider dialysis

Question 137

A 4 year old boy presents with several small bruises. He has a platelet count of 35 and a Hb of 120. A. What is the diagnosis ? B. List two management options? C. He presents a week later with recurrent nosebleeds. His platelets are now 12 and his Hb is 104. List two treatments ?

Answer 137

``` A. What is the dx? - ITP B. List two management options 1. Observe and follow up with guidance 2. Treat ``` C. Presents with bleeding. List 2 treatments 1. IVIG - 1g/kg 2. Steroids - prednisone x 4 days

Question 138

A 4 year old male comes in looking pale, tachycardic, and with abdominal pain and a large left- sided abdominal mass. Hgb 40. Sickledex screen is positive. A. How do you confirm the underlying diagnosis ? B. What is the etiology of the acute presentation ? C. What is the treatment for the acute presentation ?

Answer 138

A. Confirm dx ? - Hgb electrophoresis B. Etiology of acute presentation ? - splenic sequestration C. What is the treatment for the acute presentation ? - pain management - hydration - incentive spirometry - you can give transfusion but carefully !!!

Question 139

A 7 month old baby presents with small blue- purplish hard nodules scattered all over its skin. Urine HVA and VMA are positive. On ultrasound, the baby is found to have a small adrenal mass. A. What are 2 investigations you would proceed with to determine diagnosis and it’s categorization ? B. What is the diagnosis and what is the stage ? C. What is the prognosis ?

Answer 139

A. Two investigations 1. Bone marrow aspirate to look for small round blue cells 2. Biopsy of tumor 3. CT chest and abdomen Other: Bone scan, MIBG B. What is the diagnosis and what is the stage ? - neuroblastoma, likely stage 4S (distant spread and patient <1year) C. What is the prognosis ? - excellent, survival >90% with just supportive care

Question 140

You have a kid with spherocytosis, you get the results and the kid is slightly anemic and evidence of spherocytes on peripheral smear. A. What are two tests you can do to confirm hereditary spherocytosis ? B. What are two indications for splenectomy one this patient ?

Answer 140

A. What are 2 tests you can do to confirm hereditary spherocytosis? 1. Genetic testing 2. EMA binding test (flow cytometry) or cryohemolysis 3. Osmotic fragility test (not used anymore) B. What are two indications for splenectomy in this patient ? Usually recommended > 5 years old 1. Severe hemolytic anemia 2. Poor growth 3. Cardiomegaly due to high output cardiac failure 4. Hypoplastic or aplastic crisis

Question 141

A 13 year old boy presents with lymphoma. A. What are 5 lab abnormalities seen in Tumor lysis syndrome? B. What are three treatment options for Tumor lysis syndrome ?

Answer 141

A. 5 lab abnormalities 1. Hyperkalemia 2. Hyperuricemia 3. Hypocalcemia 4. Hyperphosphatemia 5. Elevated LDH B. 3 treatments 1. Hyper hydration 1.5x maintenance 2. Allopurinol 3. Rasburicase 4. Dialysis

Question 142

A 2 year old boy has just immigrated to Canada. He was diagnosed with sickle cell disease at 1 year. In addition to referring to a hematologist (nearest one is 500km away) and confirming the diagnosis - what are FIVE essential things to do in the first visit ?

Answer 142

Five essential things at first visit Health supervision from 1-5 years of age 1. Review disease manifestations, and treatments to date 2. Need for penicillin prophylaxis (2 mo- 5th birthday) and need to seek medical attention for febrile illness (temp >38.5C) 3. Discuss other reasons to seek medical attention - splenic sequestration, anemia, dactylitis, pain or ACS, signs of stroke or TIA 4. Review immunizations, and administer any outstanding ones like the flu vaccine, also specifically vaccines against streptococcus, meningococcus (ie; Menveo or Menactra) and hemophilus influenza 5. Baseline CBC and retic count 6. Physical exam to assess growth/development, sleep apnea, jaundice, cardio resp system, HTN, murmur, spleen size and Neuro exam 7. Consider baseline renal, liver tests, urinalysis, CXR, echo, transcranial Doppler ultrasound 8. Discuss enuresis

Question 143

Five signs/symptoms of a child with an infratentorial tumor

Answer 143

1. Symptoms of increased ICP - early morning vomiting, headache, vomiting with straining, papilledema, Cushing’s triad (increased BP, bradycardia, irregular resps) would be a late finding 2. Cranial nerve palsies/head tilt 3. Blurred vision, diplopia and nystagmus - due to effect on CN or from increased ICP 4. Signs and symptoms of ataxia and discoordination-cerebellum 5. Long tract signs like hyper reflex is, clonus, muscle spasticity, bladder difficulties, hemiparesis Tumors of the brainstem region may be a/w gaze palsy, multiple CN palsies and upper more Neuron deficits ie; hemiparesis, hyperreflexia, clonus

Question 144

Blood work consistent with HUS in a baby. A. Name 3 bugs B. What’s the pathophysiology of anemia ?

Answer 144

A. Three bugs 1. E. Coli 0157:H7 2. Shigella dysenteriae 3. Strep pneumonia B. What’s the pathophysiology of anemia? Microangiopathic hemolytic anemia - mechanics destruction of pRBCs from fibrin strand deposition in vessels.

Question 145

Kid with sickle cell disease presents in respiratory distress, CXR has 2 focal consolidation. A. What does he have ? B. Name 4 management C. What one medication would you give for prophylaxis ?

Answer 145

A. Diagnosis ? - Acute chest syndrome B. Name 4 management 1. Oxygen to keep saturation > 95% 2. Analgesics (opioids) and fluids at maintenance for hydration 3. IV abx (ceftriaxone and azithromycin) 4. Chest physiotherapy and incentive spirometry Others: if severe exchange transfusion; bronchodilators and steroids if asthma Or 1. Blood transfusion(simple or exchange)- especially when decreasing 02 sat, increased WOB, rapid change in resp effort either with or without a worsening chest radiograph, or previous history of severe ACS requiring admission to the ICU 2. Supplemental 02 for drop in pulse oximetry by 4% over baseline or values < 90% 3. Empirical antibodies (3rd gen cephalosporin and macrolide) 4. Continued respiratory therapy (incentive spirometry and chest physiotherapy as necessary ) 5. Bronchodilator and steroids for patients with asthma (even if no wheezing at the time of presentation) 6. Optimum pain control and fluid management C. One med for prophylaxis - hydroxyurea

Question 146

Exact picture above was shown (periorbital ecchymosis from neuroblastoma), what are 5 physical features ?

Answer 146

Neuroblastoma 5 physical features 1. Periorbital ecchymosis 2. Subcutaneous nodules 3. Abdominal mass (retroperitoneal or hepatic) 4. HTN 5. Opsoclonus- myoclonus-ataxia syndrome (rapid eye movements and muscle twitches ) 6. Hepatosplenomegaly 7. Limping on exam 8. Horner’s syndrome - miosis, ptosis, anhidrosis 9. Proptosis 10. Lower extremity weakness - from spinal cord compression 11. Scoliosis, bladder dysfunction 12. Systemic symptoms (fever, weight loss) 13. Heterochromia iridis (different colours of the iris or portion of the iris) 13. Unilateral nasal obstruction

Question 147

Splenomegaly is very common in the Pediatric population. A patient is diagnosed with splenomegaly in the context of an infectious mononucleosis. At what point would it be safe to recommend a return to contact sports ? (1 line )

Answer 147

Minimum of 3 weeks from symptom onset to return to contact sports, as long as the patient has complete resolution of symptoms and lab markers are resolved - ideal to look for resolution of splenomegaly by ultrasound - if unable to confirm resolution of splenomegaly by US, consider delaying return to play for another 1-2 weeks

Question 148

Name 2 long-term complications of hemophilia

Answer 148

2 long term Complications of hemophilia 1. Arthropathy and joint contractures 2. Chronic pain Other - transfusion transmitted infectious diseases - inhibitor formation (development of autoantibodies that block the activity of the relevant factor)

Question 149

Picture of a child with battle sign (dark bruises under eyes). Known to have abnormal eye movements described as dancing eyes. Give diagnosis.

Answer 149

Neuroblastoma - (opsoclonus- myoclonus- ataxia syndrome)

Question 150

Name 3 common childhood etiologies for neutropenia (aside from sepsis)

Answer 150

3 common childhood etiologies of neutropenia 1. Viral infections - EBV, parvovirus, HHV-6 2. Autoimmune neutropenia 3. Drug induced ie; side effect of antiepileptic medications, antimetabolites like methotrexate 4. Nutritional neutropenia - B12, folate, copper deficiency 5. Isoimmune neonatal neutropenia 6. Chronic idiopathic neutropenia 7. Pure white cell aplasia 8. Hypersplenism 9. Bone marrow disorders 10. Congenital neutropenia 11. Cyclic neutropenia

Question 151

5 hour old baby with petechial rash. She is well otherwise. Platelets are 9, wBC 10, Hb 170. Mother 40 year old, pregnancy normal, and healthy. What is differential diagnosis ? List 3

Answer 151

Thrombocytopenia - list 3 1. Laboratory error 2. Neonatal alloimmune thrombocytopenia 3. Neonatal autoimmune thrombocytopenia 4. Congenital disorder of diminished PLT production (ie; thrombocytopenia absent radii syndrome, Wiscott-Aldrich syndrome 5. TORCH infections (ie; CMV, rubella) 6. NEC 7. RDS 8. Maternal preeclampsia 9. Drug related 10. Hypersplenism 11. Kasabach- Merritt 12. Thrombosis (renal vein, aortic) 13 fanconi anemia 14. Amegakaryocytic thrombocytopenia

Question 152

A 7 year old girl comes to see you with decreased energy and pallor for the past week. She had a viral illness 1-2 weeks ago. You notice that her sclera seem a bit yellow. Her CBC shows a Hb 70, normal WBC, normal platelets, and a retic count of 24%. A. What type of anemia is this ? B. What test would you do to confirm ? C. What treatment could you offer ?

Answer 152

A. Type of anemia - Autoimmune hemolytic anemia B. What test would you do to confirm? - DAT (Coombs test) C. What treatment would you offer? - steroids As per jack - there is nothing in the stem that tells you it’s AIHA, but this is the only type of anemia for which there would be one test to confirm and one treatment to offer

Question 153

12 year old girl presents with pallor, scleral icterus and SOB. Exam - spleen 3cm palpable. Labs - Hb 65, WBC normal, platelets normal, total bilirubin 55g/L, direct bilirubin 3g/L. A. What is the most likely diagnosis ? B. What investigation should you use to make this diagnosis? C. What therapy would she benefit from ?

Answer 153

A. Autoimmune hemolytic anemia B. Coomb’s year, smear C. Steroids

Question 154

3 treatments for hyperuricemia from Tumor lysis syndrome.

Answer 154

1. Hyper hydration 2. Allopurinol 3. Rasburicase 4. Dialysis

Question 155

A 3 year old child presents with a 4-5 day history of fever, URTI symptoms. CBC shows a Hgb of 120, WBC 2.0, neutrophils 2% , lymphocytes 80%, monocytes 9%, eosinophils 8%, PLT 300. Name 3 management steps

Answer 155

The child has lymphopenia and significant neutropenia (ANC = 0.04), in the setting of fever Febrile neutropenia management 1. ABCs 2. Draw blood cultures, urine cultures, NPS (avoid Foley catheter for urine specimen) 3. Admit to hospital for observation under febrile neutropenia protocol 4. Broad spectrum antibiotics (piperacillin/tazobactam)

Question 156

What are 2 serious/life threatening manifestations of a mediastinal mass, and how do they present ? Question was in table form: (presentation, explanation)

Answer 156

1. Tracheal or bronchial compression: - respiratory distress - dyspnea especially when lying down - respiratory failure 2. Superior vena cava syndrome - due to compression of SVC from Tumor - resp distress - facial swelling - increased erythema of neck and face - dilated neck veins Presentation. Explanation 1. Dyspnea, orthopnea, cough, wheezing, stridor — compression of airway bronchus 2. Edema of arms/face/neck — compression of SVC 3. Hypotension — tamponade or outflow Tracy obstruction 4. Horner syndrome - compression of sympathetic chain

Question 157

Boy with edema, petechiae, etc. post gastro with bloody diarrhea. What is the diagnosis ?

Answer 157

Hemolytic uremic syndrome HUS - triad of microangiopathic hemolytic anemia, thrombocytopenia and renal insufficiency.

Question 158

A 4 year old boy has bloody diarrhea then presents with petechiae and decreased urine output. A. What is the likely diagnosis ? B. What is the pathogenesis of his anemia?

Answer 158

A. HUS | B. Microangiopathic hemolytic anemia

Question 159

7 year old boy from Kenya presents with tachypnea and chest pain. His lab work shows a hemoglobin of 40. A. What is the likely diagnosis ? B. What are four important management steps?

Answer 159

A. Sickle cell disease - acute chest crisis B. 4 important steps in management 1. ABCs 2. CXR 3. Oxygen to titrate saturations to >95% 4. Analgesics (opioids) 5. IVF (don’t overhydrate) 6. Antibiotics - 3rd generation cephalosporin + macrolide 7. Chest physiotherapy and incentive spirometry 8. Others: if severe, exchange transfusion; bronchodilators and steroids if asthma Management of acute chest crisis 1. Oxygen to titrate saturations > 95% 2. pRBC transfusion 3. Analgesics (opioids) and fluids 4. Antibiotics (ceftriaxone and azithromycin) 5. Chest physiotherapy and incentive spirometry 6. Others: if severe, exchange transfusion, bronchodilators and steroids if asthma

Question 160

A 4 year old boy presents with petechiae. He has a platelet count of 55 and a Hb of 120. A)How would you manage him ? B) He presents a week later with recurrent nosebleeds. His platelets are now 5 and his Hb is 80. What is your next step in management?

Answer 160

A.Could do one of two things: 1. No therapy other than education and counselling of the family and patient for patients with minimal, mild and moderate symptoms 2. A single dose of IVIG or a short course of corticosteroids should be used as first- line treatment B. - ABCs, CBC, smear and reticulocyte count - treat with IVIG and steroids - bone marrow aspirate to rule out malignancy - two cell lines down

Question 161

Chart with CBC findings of hereditary spherocytosis. Has been asymptomatic. What is the explanation ? (1). Now Hb 55, retic 3%, WBC 3.8, Plt 196, Bili 30. Was 3 months ago Hb 86, retic 11%, WBC 7.6, PLT 184, Bili 20.

Answer 161

Hereditary spherocytosis - aplastic anemia from Parvovirus B19 infection (explains lymphopenia, decreased reticulocytosis in the setting of anemia

Question 162

A 16 year old female was treated for high risk ALL. She received cyclophosphamide and radiation. She is premenstrual and at Tanner stage 1. She is at less than the 3rd percentile for height. A. What are the two likely causes of her delayed puberty ? B. What three investigations would you order ?

Answer 162

A. 2 likely causes of delayed puberty 1. Chronic side effect from cyclophosphamide (primary ovarian failure) 2. Radiation therapy to CNS (brain) - hypothalamic dysfunction 3. Hypothyroidism - can also be from radiation B. What 3 investigations would you order ? 1. Bone age 2. LH, FSH, estrogen, testosterone, DHEA levels 3. GnRH stimulation test if central cause 4. TSH, free T4 (hypothyroidism can occur secondary to rads) 5. Other: abdominal ultrasound to look at ovaries/uterus

Question 163

An 18 month old boy has been seen mouthing objects. He has a microcytic anemia which is unresponsive to iron therapy. His smear shows basophils stippling. What test would you order next ?

Answer 163

A. Serum lead level Lead poisening

Question 164

A baby was born with severe hydrops. She has a large renal mass. She is on maximal ventilatory and inotropic support. She has a very distended abdomen with poor perfusion to her lower limbs. Her electrolytes are within normal range. She is about to go to surgery. A. What would you tell her parents about her prognosis ? B. What are two complications you should expect in the intra-operative period ?

Answer 164

A. Congenital mesoblastic nephroma a/w hydrops fetalis is a/w a poor prognosis and is usually fatal. Poor neurodevelopmental outcome B. 2 complications intra-operatively 1. Cardioresp failure 2. Abdominal compartment syndrome

Question 165

Ways that hemophilia can present in a 1 month old.

Answer 165

Hemophilia presentation in a one month old 1. Oozing from umbilical site after the umbilical stump falls off 2. Bleeding from circumcision site 3. Intracranial hemorrhage (IVH) 4. Large caput or subgaleal hemorrhage in neonate 5. Hematoma at site of IM injection 6. Excessive bleeding with phlebotomy

Question 166

What are 3 investigations for hereditary thrombosis ?

Answer 166

3 investigations for hereditary thrombosis 1. Factor V Leiden - activated protein C resistance (mc) 2. Prothrombin gene mutation (second most common) 3. Protein C and S levels (to look for deficiency) 4. Antithrombin III 5. Homocysteine

Question 167

List one associated malignancy with each of the following: A. Aniridia B. Beckwith C. Trisomy 21 ``` Bonus ones D. Klinefelter E. Noonan F. WAS G. NF1 ```

Answer 167

List associated malignancy A. Aniridia - wilm’s Tumor B. Beckwith - wilm’s Tumor C. Trisomy 21 - acute myeloid leukemia D. Klinefelter - breast cancer, extragonadal germ cell tumors E. Noonan - JMML F. WAS - lymphoma, leukemia G. NF1- neurofibromas, optic glioma, acoustic neuroma, astrocytoma, meningioma, pheochromocytoma, sarcoma, JMML, wilm’s

Question 168

Child with microcytic hypochromic anemia. You believe it is Fe deficiency. What other features of CBC would be consistent with this? list 3

Answer 168

1. Elevated MCV/RBC count (mentzer’s index) >13 2. Increased RDW 3. Thrombocytosis Remember to look at the question - it does not ask about the smear !!

Question 169

Child with Down syndrome. List three associated hematological disorders

Answer 169

3 associated hematological disorders 1. Acute myeloid leukemia 2. Acute lymphoblastic leukemia 3. Transient myeloproliferative syndrome Up to date 1. Neonatal polycythemia 2. Children often have macrocytosis 3. Transient myeloproliferative disease 4. Acute megakaryoblastic leukemia (AMKL) - type of AML 5. ALL

Question 170

List 4 indications for blood transfusion in sickle cell disease.

Answer 170

4 indications for blood transfusion 1. Splenic sequestration 2. Aplastic crisis 3. Acute severe stroke 4. Preventative transfusions for abnormal transcranial Dopplers (stroke prevention) 5. Acute chest crisis - decreased 02 sat - increased WOB - rapid change in resp effort - prev hx of severe ACS requiring ICU 6. Priapism 7. Pre-op to increase Hb to 100

Question 171

14 year old girl with sickle cell disease presents with red swollen painful hand. No vitals mentioned. A. What are three things you do for management? B. After leaving the hospital, what is one medication that can be taken for prevention of these episodes?

Answer 171

A. Three management strategies for dactylitis - treat the same as vasoiclusive/pain crisis 1. ABCs 2. IV fluids 3. Opioids /analgesia 4. And labs/imaging (MRI hand) to rule out possible trauma and osteomyelitis 5. Incentive spirometry ( I added this) B. Hydroxyurea

Question 172

Polycythemic newborn. Hb 240, Hct 0.75. Weight 2000g. Child requires a partial exchange transfusion. A. What fluid do you use as a diligent ? B. How much blood do you replace to decrease the Hct to 0.5 ?

Answer 172

A. Normal saline B. Exchange volume = [(observed Hct - desired Hct) x blood volume] / observed Hct Weight = 2000g Observed hct = 0.75 Desired hct = 0.55 Blood volume = 80-100ml/kg baby and 80ml/kg children So for a 2kg baby x 100ml/kg = 200ml Blood volume = [(0.75 - 0.5)x 200ml]/0.75 = 66ml to exchange If you use 0.55 you get 53ml !! Not sure what they didn’t use that instead !?!

Question 173

Baby born at home at 38weeks by midwife. Now presents at 7 days with melena. Hb 35, MCV 112, PLTs normal. What is the most likely diagnosis ?

Answer 173

A. Hemorrhagic disease of the newborn (classic) Remember Early = maternal meds = phenytoin Classic = 1-4 weeks = no vitamin K at birth Late = 3 weeks to 8months = malabsorption or no vitamin K

Question 174

Sickle cell with recurrent chest crisis. What is he at increased risk of?

Answer 174

Sickle cell with recurrent chest crisis at increased risk for: 1. Recurrent cerebral infarction 2. Asthma 3. Gallstones