Peds

Question 1

Recurrent sinopulmonary infections, nasal polyps, and digital clubbing are most likely associated with what condition?

Answer 1

Cystic Fibrosis

*disclaimer: Primary Ciliary Dyskinesia has similar symptoms but less common

Question 2

What is the next step of management for a child who is living in a house built in 1940s and found to have greater than 5 micrograms/deciliter in cap blood test?

Answer 2

Draw venous lead level (cap blood results have high false positives)

Question 3

When should chelation therapy be started for lead?

Answer 3

at 45 micrograms/deciliter

Question 4

At what lead level is dimercaptosuccinic acid be used? Dimercaprol plus calcium disodium edetate (EDTA)?

Answer 4

DMSA, Succimer at 45-69; EDTA at greater than 70 or signs of acute encepholopathy

Question 5

Pancreatic insufficiency, meconium ileus, chronic metabolic alkalosis, and absence of vas deferens are signs of what?

Answer 5

Cystic Fibrosis

Question 6

How do you dx CF?

Answer 6

1) one or more symptoms, CF in sibling, or + newborn screening
2) sweat Cl greater than 60 on 2 or more occasions, id of 2 CF mutations, or abnormal nasal epithelial ion transport

Question 7

What is the gold standard dx test for CF and what method does it use?

Answer 7

Sweat testing via quantitative pilocarpine iontophoresis

Question 8

What tx for CF?

Answer 8

high calorie diet, pancreatic enzyme replacement, and fat-soluble vitamin supplementation

Question 9

How do you screen for primary ciliary dyskinesia?

Answer 9

Exhaled nasal nitric oxide

Question 10

Why do newborns have jaundice in days 2-4 of life?

Answer 10

They have unconjugated hyperbili because of

1) [RBC] (hematocrit 50-60%) with shorter life span causing high Hb turnover and bili production
2) Bili clearance is slow becuz hepatic UGT doesn’t reach adult levels until age 2 weeks (Asians have lower UGT activity)
3) more bili is reabsorbed becuz of sterile newborn gut can’t break it down to urobilinogen for fecal excretion

Question 11

This in indicated in newborns with bili levels greater than 20-25

Answer 11

exchange transfusion

Question 12

This dx test can be negative in the first few weeks after the following symptoms appear: fever, pharyngitis, fatigue, posterior cervical lymphadenopathy, palatal petechiae, splenomegaly, bilateral upper eyelid edema

Answer 12

hterophil antibody (Monospot) test
*can do EBV-specific antibodies if monospot negative

Question 13

This virus is suspected if both the Monospot and EBV specific dx test come back negative in a patient who is suspected to have mono

Answer 13

CMV

Question 14

This is the main difference between breastfeeding failure jaundice and breast milk jaundice.

Answer 14

Level of hydration (no signs of dehydration in breast milk jaundice)

Question 15

This type of jaundice is in a patient of 4 days old who has 3 wet diapers, brick red urate crystals in diaper, decreased urine and stool output, and total bili 15 with direct 0.9

Answer 15

breastfeeding failure jaundice

Question 16

Breast milk jaundice has this to cause increase of bili.

Answer 16

High levels of beta-glucuronidase which deconjugate intestinal bilirubin and increases enterohepatic circulation

Question 17

This is the best management for breastfeeding failure jaundice.

Answer 17

breastfeed 15 min per side every 2-3 hours

Question 18

This is the threshold to treat hyperbili with phototx

Answer 18

20

Question 19

This is used to tx a 2 yo who presents with a fever that began 5 days ago with URI symptoms w/o relief, subcostal retractions and scattered wheezes w/o relief, and a holosystolic murmur at the cardiac apex.

Answer 19

Supportive measures like diuretics and inotropes (for viral myocarditis)

Question 20

This steroid is most likely increased in a 1 week old female patient with a BP of 74/52, sunken eyes, enlarged clit, and fusion of labioscrotal folds.

Answer 20

17-hydroxyprogesterone (from deficiency in 21-hydroxylase)

Question 21

This is the most common cause of urinary tract obstruction in newborn boys.

Answer 21

posterior urethral valves

Question 22

This is the mechanism for Potters sequence.

Answer 22

Urinary tract anomaly leads to anuria or oliguria in utero leads to oligohydramnios leads to pulm hypoplasia, flat facies, and limb deformities

Question 23

Low birth weight, closed fists with overlapping digits, microcephaly, micrognathia, and rocker-bottom feet are signs of this.

Answer 23

Trisomy 18 (Edward’s)

Question 24

Hypotonia, flat face, upward and slanted palpebral fissures, epicanthal folds, intestinal atresia, and high arched palate are signs of this.

Answer 24

Trisomy 21 (Down)

Question 25

Cleft lip, flexed fingers with polydactyly, ocular hyptelorism, low-set ears, microphthalmia, and absent ribs are signs of this.

Answer 25

Trisomy 13 (Patau)

Question 26

An infant with a cat like cry had this genetic malformation occur to cause this syndrome

Answer 26

5p deletion (cri-du-chat)

Question 27

This most common benign vascular tumor grows rapidly and regresses spontaneously unless there are complications of which can be treated with this.

Answer 27

Beta blockers (for strawberry hemangiomas)

Question 28

This transilluminated-capable mass is associated with Turner’s, trisomy 21, 18, and 13

Answer 28

cystic hygroma

Question 29

These are blanchable, pink-red pathces on eyelid, glabella, and neck

Answer 29

nevus simplex

Question 30

The lower the birth weight, the greater the likelihood of this.

Answer 30

intraventricular hemorrhage

Question 31

Absence of pus formation at infection site, delayed umbilical cord separation, bleeding of gums, necrotic periodontal infection with high WBC with high neutrophils has a deficiency expression of this protein

Answer 31

CD 18 (Leukocyte adhesion deficiency type 1)

Question 32

Patients who present with necrotic periodontal infection and no pus formation have the inability of this type of leukocyte to travel to infected area and thus have a bx of infected tissue devoid of this.

Answer 32

Neutrophils

Question 33

This is an autosomal recessive form of SCID with deficient B and T lymphocutes and labs showing severe lymphopenia

Answer 33

Adenosine deaminase deficiency

Question 34

Patients with complement deficiencies are at risk of these type of bacterial infections.

Answer 34

Encapsulated (Strep pneumo, H influz, N. mening)

Question 35

Patient with developmental defects in pharyngeal arch, T cell deficiency, facial and cardiac anomalies, and thymic hypoplasia has this.

Answer 35

DiGeorge syndrome

Question 36

An infant with crepitus of clavicular bone, asymmetic moro reflex, and risk factors like macrosomia and shoulder dystocia should be managed with this.

Answer 36

Reassurance and gentle handling (healing usually occurs spontaneously after 7-10 days)

Question 37

An infant with jaundice, tonic-clonic seizures, hydrocephalus, chorioretinitis, and intracranial calcifications has this.

Answer 37

congenital toxoplasmosis

Question 38

An infant that gets chorioretinitis, encephalitis, and disseminated disease but no congenital anomalies

Answer 38

herpes simplex (passed through birth canal)

Question 39

An infant with deafness, cataracts, and cardiac defeats (like PDA or ASD) has this.

Answer 39

Congenital Rubella Syndrome

Question 40

Arthritis for greater than 6 weeks as well as a rash can be seen with this.

Answer 40

Juvenile idiopathic arthiritis