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Peds Shelf > Pediatrics section within UW > Flashcards

Pediatrics section within UW Flashcards

1
Q

Gold standard for duchenne diagnosis?

A

Genetic testing. Shows deletion of dystrophin gene: Xp21

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2
Q

Onset of Duchene vs Becker

A

Duchene by 2-3 years old, Becker 5-15 years old.

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3
Q

What enzyme levels are reaised in DMD?

A

Serum creatine phosphokinase and aldolase

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4
Q

Treatment for subluxation of radial head

A

Hyperpronation of forearm, supination of forearm and flexion of the elbow.

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5
Q

What is the first and second most common posterior fossa tumor?

A

1) cerebellar astrocytoma 2) medullobastoma (cerebellar vermis)

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6
Q

Posterior vermis syndrome is characterized by?

A

Truncal and gait ataxia

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7
Q

Lesch Nyan syndrome secondary to deficiency in?

A

hypoxanthine-guanine phosphoribosyl transferase (HPRT). X linked recessive disorder. Enzyme is involved with purine metabolism.

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8
Q

Sx of Lesch Nyan

A

First appears at 6 months w/ vomiting and dystonia. Then progressive mental retardation, choreoathetosis, spasticity, dysarthric speech, dystonia, self injury. Xs uric acid builds up leading to gouty arthritis, tophus formation and obstructive nephropathy

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9
Q

IF you see a boy with gout, suspect?

A

Lesch Nyan (usually gouty arhritis manifests when you’re 50 )

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10
Q

Marfan mutation of what gene

A

fibrillin 1 gene

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11
Q

What vitamin has shown to improve morbidity and mortality in measles

A

Vitamin A. through immune enhancement as well as regeneration of GI and respiratory eptihelium

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12
Q

Alpha adrenergic and beta adrenergic effects of epinephrine for asthma?

A

Alpha 1 adrenergic: vasoconstriction - increases blood pressure and decreases upper airway edema, reduces bronchial secretions, and mucosal edema. Beta 2 adrenergic: promotes smooth muscle relaxation, decreases systemic release of inflammatory mediators. These are effects of epinephrine.

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13
Q

In cases of croup, you should administer what before considering intubation?

A

Racemic epiphephrine. Reduces need for intubation

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14
Q

Metanephros is embryologic precursor for ?

A

Renal parenchyma

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15
Q

Wilms tumor arises from which embryogenic structure?

A

Metanephros

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16
Q

Three most common pediatric cancers?

A

Leukemia, CNS and neuroblastoma (TN says leukemia, CNS and lymphomas)

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17
Q

Neuroblastomas arise from which embryonic structure?

A

Neural crest cells => precursor of adrenal medulla and sympathetic chains

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18
Q

Most common location of neuroblastoma

A

Abdomen = adrenals or retroperitoneal ganglia

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19
Q

For neuroblastoma what can be seen on CT scan and plain x-ray

A

calcifications and hemorrhages

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20
Q

For neuroblastoma levels of what are elevated in serum and urine?

A

catecholamines and their metabolites (HVA and VMA)

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21
Q

Mesoneprhos

A

seminal vesicles, epidiydmis, ejaculatory ducts, and ductus deferens

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22
Q

Wilms tumor aka

A

Nephroblastoma

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23
Q

Freidrich Ataxia?

A

A/R condition. Parents should get genetic counseling for prenatal diagnosis with affected child.

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24
Q

Ppt of epiglotitis. Most concerning complication?

A

Rapid progression, drooling, sore throat, dysphagia, airway obstruction is most concerning complication.

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25
Q

Sudden LOC, disorientation, slow gain of consciousness?

A

Seizure

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26
Q

Post ictal paralysis aka

A

Todd’s paralysis

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27
Q

Todd’s paralysis

A

Post ictal condition that improves with restoration of motor function within 24 hours

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28
Q

What is micrognathia

A

Undersized jaw

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29
Q

Symptoms of Digeorge syndrome

A

CATCH 22. Cardiac anomalies (conotruncal defects, cyanotic CHD), Abnormal facies such as micrognathia and low set ears, Thymic hypoplasia (immunodeficiency, lymphopenia causing susceptibility to viral and fungal infections, poor T cell help could affect B cells and bacterial infections also), Cognitive impariment, Hypoparathyroid/hypocalcemia

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30
Q

Digeorge ngenetic abnormality?

A

Microdeltion of 22q.11

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31
Q

Recurrent infections starting at 6 months of age. Suspect?

A

Genetic defect in b cell maturation. At 6 months, maternal antibodies drop.

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32
Q

genetic defect in B cell maturation results in body inability to

A

Fight encapsulated organisms like Strep pneumo and h. influenza. Also lacks IgA and can’t fight giardia well.

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33
Q

Underlying genetic defect in SCID

A

adenosine deaminase deficiency.

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34
Q

Gold standard for CF?

A

Sweat testing by Pilocarpine iontophoresis. Pilocarpine is a cholinergic drug that induces sweating. 2 separate tests that show greater than 60mmol/L confirms dx.

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35
Q

Hallmark features of CF?

A

Failure to thrive, oily stools, recurrent respiratory infections. Defective chloride transport always involves respiratory tract, sinuses and pancreas.

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36
Q

Common hip disorder seen in overweight adolescents

A

Slipped capital femoral epiphysis

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37
Q

Gold standard treatment for slipped capital femoraph epiphsysis

A

immediate surgical screw fixation

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38
Q

Hyper IgM syndrome caused by?

A

Defect in CD40 ligand on Th2 cells, therefore can’t stimulate B cells to undergo immunoglobulin switch from B to others

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39
Q

Hyper IgM characterized by?

A

High IgM, low IgA and IgG and normal lymphocyte populations

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40
Q

Defects in humoral immune system manifested by?

A

Recurrent or severe sinopulmonary infections

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41
Q

Polycythemia can occur in SGA babies because?

A

increased RBC production in response to fetal hypoxia

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42
Q

Treatment of clubfoot should be treated immediately

A

Immediately

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43
Q

Hemophilic arthropathy?

A

Permanent joint disease due to long term consequences of hemarthrosis

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44
Q

Pathophysiology of hemophilic arthropathy?

A

iron/hemosiderin deposition leading to synovitis and fibrosis

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45
Q

Typical presentation of glucose 6 phosphatase deficiency

A

3-4 month old, hypoglycemia, lactic acidosis, hyperuricemia, hyperlipidemia, doll like face (Fat cheeks), thin extremities, short and big stomach due to hepatomegaly and big kidneys. Can also have seizures

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46
Q

Children that present with nocturnal vulvar itching should be examined for?

A

Pinworms and started on mebendazole

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47
Q

In prepubertal females, pinworm infection can present as?

A

Vulvovaginitis

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48
Q

Neonates that get chlamydia from mom can present with

A

Pneumonia (presents at 4-12 weeks with staccato cough) or conjunctivitis

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49
Q

Treatment for neonatal chlymadial conjunctivitis? Timeline (presents when)? Clinical ppt?

A

Oral erythromycin. Presents 5-14 days of life. Eyelid swelling, chemosis, and watery or mucopurulent discharge. blood tinged discharge is highly characteristic.

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50
Q

Topical erythromycin in neonates is good for?

A

prophylaxis against gonococcoal conjunctivitis and tx for other causes of bacterial conjunctivitis. DOES NOT PREVENT OR TREAT CHLAMYDIA. ORAL erythromycin is treatment of choice (oral not topical)

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51
Q

Tx for gonococcal conjunctivitis/presents when/clinical ppt?

A

aka ophthalmia neonatorum. IV or IM cefotaxime or ceftriaxone / 2-5 days / clinical ppt: marked eye swelling, profuse purulent discharge, corneal edema/ulceration (this is dangerous)

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52
Q

Myotonic muscular dystrophy - genetics?

A

A/D

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53
Q

Myotonic muscular dystrophy presentation

A

delayed muscle relaxation - grip myotonia, facial weakness, foot drop, dysphagia, and cardiac conduction anomalies. Cataracts, testicular atrophy, baldness

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54
Q

Newborns infected with chylamydia may present with

A

Conjunctivitis and pneumonia

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55
Q

Newborns infected with herpes present with?

A

Disseminated form of infection, localized CNS disease, localinzed infection of the skin, mouth and eyes

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56
Q

What is procalcitonin

A

acute phase reactant similar to CRP that is released in response to bacterial toxins

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57
Q

Sudden onset respiratory distress, no preceding illness, and focal findings on pulmonary exam in suggestive of?

A

Foreign body aspiration

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58
Q

Are chest x rays helpful in FBA?

A

Limited because object can be radiolucent

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59
Q

What is the treatment/mgmt for suspected foreign body aspiration?

A

Immediate bronchoscopy

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60
Q

B2 aka

A

Riboflavin

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61
Q

B2 found in

A

Meat, dairy, eggs, and green leafy vegetables

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62
Q

PPT of B2 deficiency?

A

Angular cheilosis, stomatitis, glossitis, normocytic normochromic anemia, seborrheic dermatitis

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63
Q

What is chemical conjunctivitis In neonate?

A

occurs in first day of life when silver nitrate is used to prevent neonatal bacterial conjunctivitis

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64
Q

Neonatal abstinence syndrome is caused by?

A

Withdrawal from opioids

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65
Q

Neonatal abstinence syndrome characterized by?

A

High pitched cry, sweating, poor feeding, irritability, tremors, seizures, tachypnea, poor feeding, vomiting, diarrhea.

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66
Q

Manifestation of leukocyte adhesion deficiency type 1

A

Absence of pus formation at infection sites, delayed cord separation (>30 days), poor wound healing, recurrent skin and mucosal bacterial infections, periodontitis, often necrotizing

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67
Q

What is leukocyte adhesion defieincy?

A

basically neutrophils can get out of vasculature and go to infection sites because they lack CD18, an essential component of integrins present on the surface of leukocytes

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68
Q

Lab results of leukocyte adhesion deficiency

A

Leukocytosis with neutrophil predominance. Cultures show staph aureus and gram neg bacilli, biopsy of infected tissue shows ifnlammatory infiltrate with no neutrophils

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69
Q

Rib notching is a specific finding for what kind of cardiac deformity?

A

Coarctation of the aorta. Caused by the dilation of the collateral chest wall vessels that forms between the hypertensive and hypoperfused blood vessels.

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70
Q

Classic triad of congenital toxoplasmosis

A

Chorioretinitis, hydrocephalus, intracranial calcifications

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71
Q

Classic triad of congnietal rubella

A

Sensoneuronal deafness, cataracts and congenital glaucoma, cardiac defects (PDA, ASD)

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72
Q

Patient with pancytopenia following drug intake, viral infection, or toxins?

A

Suspect aplastic anemia.

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73
Q

Presentation of SCID

A

Recurrent sinopulmonary infections, oral candidiasis, diarrhea, opportunisitc and viral infections

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74
Q

SCID diagnostic clinical features

A

Absent thymic shadow, absent lympho nodes and tonsils, lymphopenia, abnormal T, B1 and NK cells.

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75
Q

What is hypertelorism

A

Abnormally increased distance between two body parts.

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76
Q

5p deletion is what?

A

Cri du chat syndrome

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77
Q

Clinical ppt of cri du chat syndrome

A

cry of the cat, hypotonia, short stature, microcephaly with protruding metopic suture, hypertelorism, bilateral epicathanl folds, high arched plate, wide and flat nasal bridges

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78
Q

Trisomy 18 aka

A

Edwards syndrome

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79
Q

Trisomy 13 aka

A

Patau syndrome

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80
Q

Classic presentation for edwards syndrome

A

closed fists with 5th over 4th and index over 3rd, microcephaly, prominent occiput, micrognathia, and rocker bottom feet

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81
Q

Classic manifestation of HSP

A

Nonblanching purpura, renal involvement, GI involvement, and arthralgias/arthritis

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82
Q

Intususseption on U/S diagnosed by?

A

target sign on U/S

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83
Q

Risk of HSP?

A

GI stuff like hemorrhage or intususseption

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84
Q

CHARGE syndrome?

A

coloboma, heart defects, atresia of the choanae, renal anomalies, growth impairment, ear abnormalities/deafness

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85
Q

What is choanal atresia?

A

congenital nasal malformation = failure of the posterior nasal passage to fully canalize. CT scan shows narrowing at the pterygoid plate in posterior nasal cavity.

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86
Q

Clinical ppt of choanal atresia

A

well appearing child with intermittent cyanosis and distress when feeding that is relieved with crying.

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87
Q

Severe coughing paraoxysms can cause?

A

subcutaneous emphysema and even pneumothorax. Air leaks from the chest wall into subcutaneous tissues due to high intraalveolar pressure. Order chest x ray to rule out pneumothorax

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88
Q

Congenital lymphedema occurs due to

A

Abnormal development of lymphatic system. Leads to accumulation of protein rich interstitial fluid in the hands, feet and neck (Webbed neck in turner syndrome). This kind of lymphedema is non-pitting.

89
Q

chronic granulomatous disease should be suspected in what kind of presentation?

A

Continuous infection by catalase positive organisms, unexplained infection with serratia marascens, aspergillus, burkholderia cepacia

90
Q

chronic granulomatous disease due to?

A

Deficiency in NADPH oxidase, leading to recurrent infections with catalase positive organisms

91
Q

Dx of chronic granulomatous disease is by

A

Nitroblue tetrazolium slide test, flow cytometry, cytochrome C oxidation

92
Q

Common complication of sickle cell: right hip pain, gradual, restriction of abduction and internal rotation

A

Aseptic necrosis of the femoral head due to occlusion of end arteries supplying femoral head, bone necrosis, and eventual collapse of periarticular bone and cartilage

93
Q

Genu varum

A

Outward bowing of legs

94
Q

Cephalohematoma

A

Subperiosteal hemorrhage, no discoloration of overlying scalp, takes a few hours to develop because it’s a slow process. Doesn’t cross suture lines. Most cases resorb spontaneously within 2 weeks - 3 months.

95
Q

Caput succadeneum

A

diffuse, sometimes echymotic swelling of the scalp. May extend across midline and cross suture lines

96
Q

Erb duchenne palsy aka? What nerves get affected?

A

Waiters tip. Extended elbox, pronated forearm, flexed wrists and fingers, intact grasp reflexes. C5 and C6

97
Q

Shoulder dystocia compliations

A

Fractured clavicle, fractured humerus, erb-duchenne palsy, Klumpke palsy

98
Q

Klumpke palsy aka? What nerves can be affected and what ensues if this is the case?

A

Claw hand. C8 and T1. you can get horners sydnrome.

99
Q

Pathognominic facial features for in utero exposure to alcohol

A

Small palpebral fissures, thin lip, smooth filtrum. Also microcephaly

100
Q

Key physical findings for Fragile X and Downs syndroem

A

Fragile X: macroorchidism, long narrow face, prominent forehead and chin, large ears, macrocephay. Downs syndrome: low set ears, upslanting palpebral fissures, flat facial profile, may have increased neck skin, single transverse palmar crease, clinodactyly, large space between first two toes.

101
Q

Double bubble on xray

A

Duodenal atresia

102
Q

Triple bubble and gasless colon on xray

A

Jejunal atresia

103
Q

Distended loops of bowel on xray

A

Hirschsprung

104
Q

Hallmark finding on x ray of NEC?

A

Pneumatosis intestinalis (extravasation of gas into damaged bowel wall)

105
Q

Jejunal atresia is due to?

A

Thought to occur due to vascular accident in utero that causes necrosis and resorption of the fetal intestine, sealing off and leaving behind blind proximal and distal ends of the intestine

106
Q

In contrast to duodenal atresia, jejunal and ileal atresia are not a/w?

A

Chromosomal abnormalities

107
Q

Risk factors for jejunal atresia

A

Cocaine and other vasoconstrive events/drugs

108
Q

Confirmatory tests for SLE?

A

Anti smith antibodies and anti dsDNA antibodies

109
Q

Coomb’s test is dx test for?

A

Autoimmune hemolytic anemia

110
Q

What test is more specific for diagnosis of syphilis?

A

FTA (fluorescent treponemal antibody test)

111
Q

Next step for battery lodged in esophagus and why?

A

Immediate endoscopic removal to prevent esophageal ulceration and mucosal damage. If beyond that, usually observe and or follow up with X ray and see if it passes uneventfully.

112
Q

Risk factors for shoulder dystonia

A

Maternal diabetes resulting in fetal macrosomia

113
Q

Risk factors for neonatal displaced clavicular fracture

A

fetal macrosomia (maternal GD, post-term pregnancy), instrumental delivery (vacuum, forceps), shoulder dystocia

114
Q

Triad of hemolytic uremic syndrome? PPT?

A

Acute renal failure, hemolytic anemia and thrombocytopenia. Suspect in patient who shows up with abdominal pain and diarrhea, followed by bloody diarrhea

115
Q

HUS due to?

A

E coli O157:H7 toxin or shiga toxin (for majority cases. Can also be due to strep pneumo, viruses or drugs)

116
Q

Pathophysiology of HUS?

A

Toxin binds, invades and destroys colonic epithelial cells, causes bloody diarrhea. Toxin enters systemic circulation, attaches and injures endothelial cells especially in kidney, and also releases endothelial cell products including platelet aggregating factor. Forms platelet/fibrin thrombi in multiple organs causing thrombocytopenia. RBCs are forced to enter occluded vesses resulting in fragmented RBCs and removed by the reticuloendothelial system (hemolytic anemia).

117
Q

What is opisthotonos

A

Spasm of muscle causing backward arching of the head, neck and spine.

118
Q

Neonatal tetanus is generally seen in what situations

A

Babies born to unimmunized mothers, following umbilical cord/stump infection.

119
Q

Neonatal tetanus presents how?

A

Poor suckling and fatigue, followed by ridigity spasms and opisthotonos

120
Q

Most common heart disease in Edwards syndrome? (Trisomy 18)

A

VSD

121
Q

Galactosemia presentation

A

Failure to thrive, poor feeding, jaundice, hepatosplenomegaly, cataracts

122
Q

Galactosemia?

A

a/R deficiency in galactose 1 phosphate uridyltransferase, leading to inability to process lactose/galactose.

123
Q

Galactosemia treatment?

A

Elimination of galactose in diet. Most infants fed soy diet.

124
Q

B1 aka

A

Thiamine

125
Q

B3 aka

A

Niacin

126
Q

B6 aka

A

Pyrodixine

127
Q

B9 aka

A

Folate, folic acid

128
Q

B12 aka

A

Cobalamin

129
Q

What race boys are increased risk for fetal macrosomia (>4 kg)

A

Black boys

130
Q

What is the tx and prognosis for Erb-Duchenne palsy from shoulder dystocia?

A

Tx: gentle massage and PT to prevent contractures. Prognosis depends on extent of nerve damange (C5, C6 and maybe C7 in Erb duchenne). Up to 80% of patients have spontaneous recovery within 3 months. If no improvement by 3-6 months, consider surgery

131
Q

Upper motor neuron signs

A

Hyperreflexia, babinski, leg spasticity

132
Q

Atlantoaxial instability presentation?

A

Sx occur due to compression of the spinal cord. Behavioral changes, torticollis, urinary incontinence, vertebrobasilar sx such as dizziness, vertigo and diplopia. UMN signs such as babinski, leg spasticity, hyperreflexia

133
Q

Classic ppt of craniopharyngioma

A

Symptoms of ICP (headaches, vomiting), calcified lesion above the sella, bitemporal hemianopsia, presence of calcified parasellar lesion on MRI is almost pathognominic

134
Q

Common associations with celiac disease?

A

Type 1 diabetes, dermatitis herpetiformis, poor absorption = iron deficient anemia

135
Q

Diagnostic tests for celiac disease?

A

IgA anti-tissue transglutaminase antibody assay, upper GI endoscopy with small intestinal biopsy

136
Q

Acute stroke syndrome after foreign body injury to soft palate likely due to?

A

Internal carotid artery compression (causes thrombosis that embolizes to brain and causes stroke) or dissection of internal carotid artery that causes ischemic stroke.

137
Q

Solitary, painful, lytic long bone lesion with overlying swelling and hypercalcemia differential?

A

Langerhans cell histiocytosis and other neoplastic processes

138
Q

Freidrich Ataxia ppt

A

1) neurologic (dysarthria, ataxia) 2) skeletal (scoliosis, foot deformities like hammer toes) 3) cardiac (concentric hypertorphic myopathy)

139
Q

Most common causes of death

A

Cardiac and respiratory

140
Q

In esophageal atresia with TEF, what does x ray show in terms of the naso gastric tube or oro gastric tube

A

tube will be in proximal esophageal pouch and can’t go any further

141
Q

If newborn chokes and coughs during first feeding, suspect?

A

Esophageal atreasia with TEF

142
Q

Kids with esophageal atresia with TEF can develop what lung disease?

A

Aspiration pneumonia. Gastric fluid refluxes through distal esophagus into fistula into trachea and lungs

143
Q

2 year olds and 3 year olds should build a tower of how many cubes

A

6 cubes and 9 cubes, respectively

144
Q

Spirochete responsible for lyme disease

A

Borrelia burgdorferi from the ixodes tick

145
Q

Breastfed infants are at decreased risk for

A

Otitis media, Gastroenteritis, respiratory illnesses, urinary tract infections and necrotizing enterocolitis

146
Q

Contraindications to breastfeeding (on infant side)

A

galactosemia

147
Q

immunofluorescence will show what in HSP?

A

IgA deposition in the mesangium

148
Q

Herpangina clinical ppt

A

High fever and sore throat that may result in inability to swallow, sometimes necessitates IV hydration

149
Q

Herpangina is caused by?

A

Throat infection caused by enteroviruses, especially coxsackie A

150
Q

Risk factors for vitamin D deficiency

A

Darker skin, no sunlight exposure, breast fed babies

151
Q

PPT of vit d deficiency

A

Genu varum, rachitic rosary, cupping and fraying of metaphyses, craniotabes

152
Q

Indirect hyperbilirubinemia in the newborn is due to?

A

Increased bilirubin production, decreased bilirubin clearance, and increased enterohepatic recycling

153
Q

Spondylolisthesis

A

development disorder caused by slipped forward vertebrae (usually L5 over S1). Results in chronic back pain and neurologic dysfunction.

154
Q

spondylolisthesis ppt

A

back pain, neurologic dysfunction and step off at lumbosacral area

155
Q

Which primitive reflex can persist in healthy children up to 2 years of age?

A

Babinski. May disappear in some by 12 months but can persisit till 2 years

156
Q

Development dysplasia of the hip should be checked for by what maneuvers?

A

Barlow and Ortolani maneuvers.

157
Q

TOC for babies 6 months and below for DDH?

A

Pavlik harness that keeps hips flexed and kneeds abducted.

158
Q

Indication for ultrasonography or x ray after barlow and ortolani test?

A

If negative tests but these findings: Soft click, or leg length discrepancy or extended inguinal fold should promot ultrasound in less than 6 months and x ray in greater than (4 or greater than 6 months of age.)

159
Q

Positive barlow and ortolani tests, next step?

A

Refer to peds ortho

160
Q

What supplementation should be given to preterm infants

A

Iron, till 1 year of age

161
Q

Alternative to venous catheterization in case of emergencies

A

Intraosseous cannulation. Preference for tibia since it’s away from cardiac site incase rescuscitation needs to take place.

162
Q

Thymus on X-ray of kid below 3 years of age

A

TRIANGULAR SHAPE ON THE RIGHT. RECOGNIZE IT. Referred to as the sail, has scalloped border and uniform density

163
Q

LOCATION Of thymus

A

In anterior mediastinum, behind the sternum and in front of the heart, aortic arch and trachea

164
Q

What happens to thymus at puberty

A

Thymus is usually replaced by fat after puberty, after production of T lymphocytes. Adults with mediastinal opacities should undergo further workup for pathology. Residual thymic tissue can undergo malignant transformation

165
Q

Infants and kids >1 year and adults have cardiothoractic ratios of?

A
166
Q

Classic triad of Kartageners

A

Recurrent sinuisits, bronchiectasis, and dextracardia

167
Q

Primary issue in Kartageners

A

Cilia dysmotility. A/R condition

168
Q

Remember that hearing impairment can look like developmental and behavioral disorders.

A

Remember that ADHD does not have social isolation or language developmental delays and that autism signs genearlly show up before 3 years of age.

169
Q

Clinical ppt of measles

A

cough, conjunctivitis, coryza (inflammation of membranes lining the noise) and KOPLCK SPOTS ARE PATHOGNOMONIC

170
Q

Dx of measles

A

Acute and convalescent serology of anti-measles IgM and IgG, polymerase chain reaction

171
Q

Rubella vs Measles rash

A

Both spread cepahlo-caudally but the measles rash darkens whereas the rubella one doesn’t

172
Q

What is the most common anemia in premature and low birth weight infants?

A

Anemia of prematurity

173
Q

Ppt of anemia of prematurity

A

Poor feeding, poor weight loss, tachypnea, tachycardia, pallor

174
Q

Pathology of anemia of prematurity

A

Decreased RBC production, blood loss, and shortened RBC life span.

175
Q

What does iron supplementation do in anemia of prematurity

A

It doesn’t help the anemia right now because iron deficiency is not the cause of the anemia but it Is given so that there are stores for later erythropoeisis.

176
Q

Immediate steps in patient with CDH?

A

Intubation and placement of gastric tube to prevent distention of bowel against the lungs/decompression the stomach and bowel.

177
Q

Conjugated hyperbilirubinemia and hepatomegaly in neonate should raise suspicion for?

A

Biliary atresia

178
Q

First step in evaluation of biliary atresia? What does it show?

A

Ultrasound. Absent or abnormal gallbladder

179
Q

Treatment for biliary atresia

A

Kasai procedure (hepatoportoenterostomy) and liver transplant

180
Q

Presentation of biliary atresia

A

Conjugated hyperbilirubinemia, pale stools, dark urine, hepatomegaly, jaundice. Initially well appearing but starts developing symptoms in 1-8 weeks.

181
Q

In older children with recurrent intussusecption, what should be suspected?

A

Pathological lead point like Meckel’s diverticulum

182
Q

Leukocoria is what unless proven otherwise

A

Retinoblastoma. Refer to ophthalmologist

183
Q

What are contraindicaitons to breastfeeding on moms side

A

Active substance use, chemo/radiation therapy, herpetic breast lesions, active untreated TB (they can breastfeed after 2 weeks of initiation of TB therapy), materinal hiv infection in developed countries, varicella infection

184
Q

Acute otitis media characteristics?

A

Middle ear effusion and signs of eardrum inflammation. Fluid in the middle ear space limits eardrum mobility on pneumatic insufflation. Bulging eardrum most specific sign of eardrum inflammation.

185
Q

Intraventricular hemorrhage is most commonly seen in?

A

Premature or low birth weight babies

186
Q

Presentation of intraventricular hemorrhage babies?

A

Pallor, cyanosis, hypotension, seizures, focal neurologic signs, bulging or tense fontanel, apnea and bradycardia. However may be asymptomatic so newborns with predisposing factors need fontanel U/S

187
Q

Incidence of IVH is inversely proportional to?

A

Inv proportional to birth weight

188
Q

Applying silver nitrate to the eyes of newborn

A

Neonatal gonococcal opthalmia prevention

189
Q

Fragile X phenotype

A

Prominent jaw, forehead, and nasal bridge with long and thing face, large protuberant ears, macroorchidism, hyperextensibility, high arched palate. Mild to moderate mental retardation, especially in boys

190
Q

How does squatting improve TOF cyanosis?

A

Increases systemic resistance, decreases the right to left shunt and increases outflow through the pulmonary artery

191
Q

What is metatarsus adductus

A

congenital foot deformity. There are 3 grades. First grade, overcorrects in abducted position. This one corrects spontaneously. Just reassurance to parents

192
Q

what is waterhouse friedrichson syndrome. When can it present?

A

Fulminant vasomotor collapse and skin rash (large purpuric lesions on flank). Watch out for it in Waterhouse Friedrichson Syndrome.

193
Q

Most common offending pathogens for AOM?

A

Bacterial - S. pnemoniae (25-40%), untypable Haemophilis influenzae (10-30%), Moraxella catarrhalis (5-15%)

194
Q

Tx for AOM?

A

Empiric amoxicilin tid x 10days, second line amoxicillin-clavulanic acid x10day

195
Q

RF for AOM?

A

cigarette smoke, allergic rhinitis, URI, craniofacial abnormalities, chronic middle ear effusion

196
Q

Phenytoin side effects

A

Cardiac defects: VAPAC (VSD, ASD, PS, AS, coarctation), craniofacial anomalies, fingernail hypoplasia, growth deficiency, developmental delay, facial clefts

197
Q

APGAR Scoring system. What score requires resuscitation

A

If under 7 may require resuscitation.

198
Q

APGAR

A

Appearance, Pulse, Grimace, Activity, Respiratory Effort. Appearance: 0 -blue/pale 1 - Acrocyanosis 2 - pink all over; Pulse (Heart rate) - 0 - Absent 1 - 100 bpms; Grimace (Irritability): 0 - No response 1 -grimace 2 - cough/cry; Activity (tone): 0 - limp 1 - some flexion of extremities 2- active motion; Respiratory effort: 0 - absent 1 - slow, irregular 2 - good, crying.

199
Q

In peds, locations of the most common CNS tumors?

A

60% infratentorial (cerebellum, midbrain, brainstem), 25% supratentorial

200
Q

Most common CNS tumor histology

A

Astrocytomas for both infra and supra-tentorial.

201
Q

Treatment for Kawasaki disease?

A

Aspirin and IVIG

202
Q

Most serious complicaitons of Kawasaki

A

Coronary aneurysms, MI, and death

203
Q

Presentation of cholesteotoma

A

Continued ear drainage despite abx. Leads to formation of retraction pocket in tympanic membrane that can be filled with granulation tissue and skin debris.

204
Q

Most common cause of hip pain in children

A

Transient synovitis

205
Q

How is transient synovitis treated?

A

Rest and ibuprofen. Usually no lab abnormalities

206
Q

Bilateral hip xrays should be obtained in transient hip synovitis for?

A

Legg calve perthe disease.

207
Q

What is legg-calves-perthes disease?

A

Characterized by insidious onset of hip or knee pain. Avascular necorosis of the femoral head. Classically affects males 4-10.

208
Q

What happens in PKU?

A

Phenylalanine hydroxylase deficiency prevents conversion of phenylalanine to tyrosine leading to buildup of toxic metabolites that cause neurologic injury.

209
Q

PKU presentation

A

Hypopigmentation (fair skin), seizures, mental retardation, eczema, mousy odor

210
Q

Dx of PKU

A

Newborn screening (Tandem mass spectrometry) or if done later, quantitative amino acid analysis.

211
Q

Treatment for PKU

A

phenlyalanine free diet, starting within first 10 days of life

212
Q

Diamond backfan syndrome aka

A

Congenital hypoplastic anemia

213
Q

Primary pathology in diamond backfan syndrome

A

Defect in erythroid progenitor cells leading to increased apopotosis

214
Q

PPt for diamond backfan syndrome

A

Pallor. Macrocytic anemia, low retic count and congenital anomalies, short stature (webbed neck, cleft lip, shield trest, triphalangeal thumbs).

215
Q

Howell Jolly bodies?

A

Nuclear remnants of RBCs. These are tradiotionally removed by spleen but patient with sickle sell with splenectomy or functional asplenia will have these in peripheral smear

216
Q

Basophillic stippling

A

Ribosomal precipitates which appear as blue granules of various sizes. Disperpsed throughout cell cytoplasm. Seen in thalassemia, lead and heavy metal poisoning

217
Q

Helmet cells

A

Fragmented RBCs. Can be seen in traumatic hemolytic conditions like DIC, HUS, TTP

218
Q

Reduction/tx of radial head subluxation

A

supination with flexion at elbow, or hyperpronation of forearm

219
Q

Red cell distribution width in iron deficiency anemias and thalassemias

A

RDW is generally greater than 20% in iron deficiency anemia, 12-14% in thalassemias

Peds Shelf (12 decks)

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