6th Year Exam

Question 1

Antibiotics in mononucleosis

Answer 1

Amoxicillin can cause rash

Cephalosporin will not give rash

Question 2

10 poisons in childhood

Answer 2

1) acetaminophen
2) salicylate
3) oral hypoglycemics
4) anticholinergics
5) organophosphates
6) caustic ingestion (acid/alkali)
7) opioids
8) acute iron poisoning
9) lead poisoning
10) ethanol

Question 3

Examples of AD inheritance

Answer 3

Achondroplasia, Ehler-Danlos, Marfan, MEN syndromes, NF1 and2, osteogenesis imperfecta, vWf deficiency

Question 4

Examples of AR inheritance

Answer 4

AR polycystic kidney diseases, CF, galactosemia, hereditary hyperbilirubinemias, maple syrup urine disease, phenylketonuria, some primary immunodeficiencies,

Question 5

Examples of XR inheritance

Answer 5

G6PD deficiency, Bruton agammaglobulinemia, Hemophilia A and B, Duchenne and Becker

Question 6

Examples of XD inheritance

Answer 6

Fragile X syndrome, Alport syndrome, x-linked hypophosphatemic rickets

Question 7

Short stature calculation

Answer 7

Mid-parental height = (fathers height + mothers +/- 13cm)/2

+boys/-girls

Question 8

Quick about changes in circulation at birth

Answer 8

Dec. pulmonary resistance -> inc pressure L atrium -> Foramen ovale close -> inc O2 from resp and dec in PGs from placental separation -> closure of ductus arteriosus

Question 9

Synonym for congenital hypoplastic anemia

Answer 9

Diamond blackfan anemia

Question 10

Viruses connected to aplastic anemia

Answer 10

Parvovirus B19, HIV, hepatitis, EBV, CMV

Question 11

Primary amenorrhea - ovarian issues

Answer 11

• Turner syndrome (streak gonads)
• Aromatase def. (lack hormone to convert androgen to estrogen)
• Chemo/radiation (ovarian failure)

Question 12

Primary amenorrhea - hypothalamus/pituitary issues

Answer 12

• Kallmann syndrome (Failure of GnRH-producing cells to develop, also anosmia)
• Tumor (compression/infiltration of pituitary)
• Malnutrition (leads to hypothalamic dysregulation, inhibits GnRH)

Question 13

Causes of primary testicular failure

Answer 13

Klinefelter (XXY), cryptorchidism, mumps orchitis, injury, chemo/radiation

Question 14

Causes of secondary testicular failure

Answer 14

Pituitary disorders, inflammatory diseases, Kallmann syndrome, drugs, obesity

Question 15

Intestinal atresia

Answer 15

Failure of a hollow viscus organ to develop appropriately. Can be stenosis, absence or rotation of a portion of the intestine

Question 16

Sign duodenal atresia

Answer 16

Double bubble sign on x-ray (also seen in malrotation with volvulus)

Question 17

Sign pyloric stenosis

Answer 17

String sign (barium studies)

Question 18

Tumors of soft tissue and bone

Answer 18

Rhabdomyosarcoma, osteosarcoma, Ewing sarcoma

Question 19

Osteosarcoma predilection sites + signs on imaging

Answer 19

Metaphysis of long bones. Codman triangle, sunburst sign.

Question 20

Ewing sarcoma predilection sites + signs on x-ray

Answer 20

Flat bones of axial skeleton (+diaphysis of long bones). Onion skin appearance

Question 21

Benign bone tumors

Answer 21

Osteochondroma (long bones), osteoma (facial bones), osteoblastoma (vertebrae)

Question 22

Diseases with fever and rash

Answer 22

Measles, Rubella, 5th disease (Parvovirus B19), 6th disease (HHV-6), Varicella zoster, scarlet fever, systemic JIA, Kawasaki

Question 23

Definition infantile respiratory distress syndrome (IRDS)

Answer 23

(Previously called hyaline membrane disease). Lack of pulmonary surfactant resulting in mild to severe respiratory compromise in neonatal period.

Question 24

Examples of chromosomal abnormalities

Answer 24

Turner syndrome, Down syndrome, Edwards (18), Patau (13), Prader-Willi syndrome, Angelman syndrome

Question 25

Diseases with fever w/o rash

Answer 25

Usually bacterial infection in babies <3months, usually viral in babies >3 months

Question 26

BPD definition

Answer 26

Oxygen requirement > 28 days, or at 36 weeks postmenstrual age in premature infant with characteristic radiologic, clinical and pathological findings.

Question 27

Retinopathy of prematurity

Answer 27

Retinal vessels grow into vitreous, causing retinal detachment and blindness if severe (90% of babies <750g, 50% if 1000-1250g)

Question 28

Diseases with chronic fever (examples)

Answer 28

EBV, CMV, TB, HIV, rickettsiae, Kawasaki, JIA, SLE, malignancy

Question 29

Newborn rashes

Answer 29

- Milia: keratin-filled cysts (face) - Sebaceous gland hyperplasia: due to maternal androgens (face) - Erythema toxicum: 2-3cm w/central tiny vesicle/pustule - Acne neonatorum

Question 30

How are infections spread perinatally?

Answer 30

Vertical (transplacental), ascending from vaginal canal, during delivery

Question 31

Perinatal bacterial infections

Answer 31

GBS, Listeria, E.coli, N.gonorrhea, C.trachomatis, T.pallidum

Question 32

Criteria for infective endocarditis

Answer 32

Duke: Major: blood culture pos. for typical organisms, echo showing valvular vegetation Minor: predisposing cardiac lesion, IV drug use, temp >38, embolic phenomena, immunologic phenomena, pos. blood culture not meeting above criteria (Definite: 2 major OR 1 major + 2 minor)

Question 33

Etiology of myocarditis

Answer 33

Usually viral (especially coxsackie B or adenovirus)

Question 34

Definition recurrent abdominal pain

Answer 34

Pain more than 1x/month for more than 3 months that interferes with daily functioning

Question 35

Red flags abdominal pain

Answer 35

Awakened by pain, ass. fever or weight loss, bloody stool, emesis

Question 36

Acquired immunodeficiencies

Answer 36

HIV/AIDS, sickle cell disease, malignancies, immunosuppression by medication, chemotherapy, malnutrition

Question 37

Disorders of amino acid metabolism

Answer 37

1) Phenylketonuria 2) Maple syrup urine disease 3) Homocysteinuria 4) Tyrosinemia

Question 38

Conjugated hyperbilirubinemia (causes)

Answer 38

1) Rotor and Dubin-Johnson 2) Alpha-1 antitrypsin def. 3) Niemann-Pick and Gaucher 4) Infections/systemic diseases causing cholestasis 5) Obstruction: biliary atresia, CF, masses

Question 39

Unconjugated hyperbilirubinemia (causes)

Answer 39

1) Hemolytic anemias | 2) Gilbert and Crigler-Najjar

Question 40

Causes of encephalitis

Answer 40

Bacterial: Bartonella, Borrelia, Mycoplasma, Rickettsia Viral: HSV, enteroviruses, CMV, EBV

Question 41

Congenital disorders of carbohydrate metabolism

Answer 41

1) Glycogen storage diseases 2) Galactose metabolism disorders 3) Hereditary fructose intolerance