Pediatrics Flashcards
(244 cards)
1
Q
What is lymphadenitis, and the most common cause?
A
An enlarged, tender and erythematous LN.
Acute unilateral – bacterial infection, norm S. aureus and GAS #2. Often febrile in kids <5y, with node >5cm
MAC is a common cause of unilateral subacute/chronic lymphadenopathy (normally nontender, afebrile, and has thin overlying violaceous skin). Kids <5y w/node <5cm
2
Q
Presentation of Langerhans cell histiocytosis:
A
Lytic bone lesions (skull, jaw, femur) seen in diaphysis of long bones
Skin lesions (purplish papules, eczematous rash)
Lymphadenopathy, hepatosplenomegaly
Pulmonary cysts/nodules
Central DI
3
Q
First step in evaluating primary amenorrhea:
A
Pelvic US
4
Q
Gold standard for dx of Muscular Dystrophy:
A
Genetic testing
5
Q
What are contraindications to admin of the DTaP?
A
Anaphylaxis, unstable neurologic disorders, and encephalopathy (coma, decreased consciousness, prolonged seizures).
Uncomplicated seizures aren’t a contraindication
6
Q
What radiologic signs are characteristic of pyloric stenosis?
A
String sign on barium swallow/upper GI series
Shoulder sign – filling defect in antrum d/t inward prolapse of mm.
Mushroom sign – hypertrophic pylorus against duodenum
Railroad track sign – excess mucosa in pyloric lumen, gives 2 columns of barium
7
Q
Metabolic disturbance seen in pyloric stenosis:
A
Hypochloremic, hypokalemic, metabolic alkalosis – d/t vomiting
8
Q
CHARGE Syndrome:
A
Coloboma -- defects in the lens, iris or retina Heart Defects Atresia choanae Retardation of growth/development Genito-urinary anomalies Ear abnormalitites/deafness
9
Q
Tx of choice for foreign body ingestion:
A
Flexible endoscopy – can visualize and retrieve object.
If the object is non-toxic and smooth in a child that is asx, 24hr observation may be done first.
10
Q
Features and risk-factors for Milk/Soy-protein-induced colitis:
A
Vomiting/Regurg by 2-8 weeks
Often have painless bloody stools and eczema as well.
Risk Factors: Fhx of allergies, eczema or asthma
Spontaneously resolves by 1 year
11
Q
What is a diffusely narrow colon a/w failure to pass meconium likely a result of?
A
Cystic Fibrosis
12
Q
When do you see microvesicular fatty changes in the liver?
A
In Reye syndrome
Macrovesicular fatty changes are seen in EtOH liver disease and NASH.
13
Q
What is tethered cord syndrome and what is it a/w?
A
It is a syndrome of weakness, decreased sensation, urinary incontinence, and hyporeflexia.
The cord is affected below T12/L1 and therefore UMN sxs are not seen
Commonly a/w Spina bifida.
14
Q
What are common sxs seen after traumatic injury to the carotid aa.?
A
May get hemiparesis, facial droop, and aphasia d/t dissection or thrombus formation which may occur over hours or days.
Neck pain, thunderclap HA, and sxs of ischemic stroke are also common.
Often d/t penetrating trauma, seemingly minor oropharyngeal trauma, or neck strain/manipulation.
15
Q
Most common source of infection for Staphylococcal Scalded Skin Syndrome (SSSS) in neonates:
A
The umbilicus or circumcision site.
16
Q
Most common source of infection for Staphylococcal Scalded Skin Syndrome (SSSS) in older children:
A
Nasopharyngeal colonization or a primary skin lesion (pustule).
17
Q
Common abnormal PE findings a/w strabismus (6):
A
Constant strabismus at any age Eye deviation after 4mo of age Asymmetric corneal light reflexes Asymmetric intensity of red reflexes Deviation on cover test Torticolis or head tilt
18
Q
Gold standard for diagnosing VUR:
A
Voiding cystourethrogram (VCUG). Should only be done in cases of recurrent UTIs, not after the first UTI as this can lead to false negative.
19
Q
Clinical features of Iron poisoning:
A
W/in 30min-4d: AbdP, Vomiting (hematemesis), diarrhea (melena), HoTive shock, anion gap Metabolic acidosis.
w/in 2d: Hepatic necrosis
W/in 2-8 weeks: Pyloric stenosis
**See Radiopaque pills on XR
20
Q
Features of Cephalohematoma:
A
Subperiosteal hemorrhage occurring d/t birth trauma.
Appears as a scalp swelling that is firm, nontender, does not cross suture lines, and no skin discoloration.
Often not seen until several hrs after birth.
21
Q
Features of Caput succedaneum:
A
Scalp swelling superficial to the periosteum that crosses the suture lines.
Edema is usually in the portion of the head that presents during vertex delivery and is present at birth.
22
Q
What is the post exposure pphx for pertussis?
A
Macrolides for all close contacts
<1mo: Azithromycin x 5d
>1mo: Azithromycin x 5d, Clarithromycin x 7d, or
Erythromycin x 14d
23
Q
Mgmt for Androgen Insensitivity Syndrome:
A
Elective gonadectomy (for malignancy prevention) and gender identity/assignment counseling.
24
Q
What is hyposthenuria and what are its sxs?
A
Inability of the kidneys to concentrate urine.
The hypoxic, hyperosmolar conditions in the renal medulla cause RBCs to sickle in the vasa recta and impairs free H2O reabsorption and countercurrent exchange.
Sxs: polyuria and nocturia despite fluid restriction.
Urine osmolality is low with normal serum Na+.
Often seen in SCD and SCT
25
Complications of Sickle Cell Trait:
```
Hematuria/papillary necrosis
Hyposthenuria
Splenic infarction (at high Alts), Venous thromboembolisms
Priapism
Exertional rhabdomyolysis
```
26
What is erythema toxicum neonatorum?
An asymptomatic/benign rash. Often scattered, erythematous papules and pustules.
Does not need diagnostic workup or treatment.
27
Contraindications to Rotavirus vaccine:
Anaphylaxis
Hx of Intussusception or uncorrected congenital Malformation of the GIT (meckel’s etc)
SCID
28
CoD in different types of muscular dystrophy:
Duchenne – respiratory failure by 20-30yr
Becker – heart failure by 40-50 yr
Myotonic dystrophy – either one depending on age of onset
29
Fanconi syndrome:
Type 2 RTA, Glucosuria, aminoaciduria and phosphaturia
30
Features of Type 1 RTA:
Poor H+ secretion into the urine – alkalotic urine (pH>5.5)
Will have low-normal serum K+
Often a genetic disorder a/w nephrolithiasis.
Can be 2/2 Rx toxicity, or AI disorders (Sjogren, RA)
31
The primary defect in different types of RTA:
Type 1 – poor H+ secretion into urine
Type 2 – poor HCO3 resorption
Type 4 – aldosterone resistance
Type 1 will have alkalotic urine, types 2 and 4 will be acidotic
32
EMG patterns in different types of muscular dystrophy:
Both DMD and BMD display a myopathic pattern on EMG.
| Myotonic dystrophy will display a myotonic pattern.
33
What is the primary cause of abnormal uterine bleeding in adolescents?
Immaturity of the Hypothalamic-pituitary-ovarian axis
| Tx w/ progestin only or combined OCs to help regulate menses.
34
When should an infant be responding to its own name?
By 12 months. If it doesn’t happen by then, it may be an early sign of autism.
35
XR findings of Ewing Sarcoma (4):
Central lytic lesion
“Onion skinning” (lamellated periosteal rxn)
“Moth-eaten” appearance
Periosteal elevation (Codman triangle)
36
Typical EM findings of Alport’s syndrome:
Alternating areas of thinned and thickened capillary loops w/splitting of the GBM.
37
Pathophys of Guillain-Barre:
Immune-mediated demyelination of peripheral nerve fibers
38
Tx of slipped capital femoral epiphysis:
Prompt surgical pinning of the slipped epiphysis where it lies – will lessen the risk of avascular necrosis and chondrolysis.
39
First step in evaluating precocious puberty:
Determine bone age
40
How to differentiate causes of precocious puberty w/Normal bone age:
Isolated breast development – premature thelarche
| Isolated pubic hair development – premature adrenarche
41
How to differentiate causes of precocious puberty w/advanced bone age:
Low basal LH and low LH after GnRH stimulation test – peripheral precocious puberty (McCune)
High or low basal LH plus high LH after GnRH stimulation – central precocious puberty
42
Features and causes of central precocious puberty:
Advanced bone age, elevated FSH & LH, and true precocious development (breast development etc)
Hypothalamic glioma
Pituitary hamartoma
Idiopathic precocious puberty
43
What is premature adrenarche?
Caused by early activation of adrenal androgens, more common in obese children.
Presents w/precocious development of pubic and axillary hair, acne, and body odor w/normal bone age.
44
What is pathognomonic for abusive head trauma in a child?
Retinal hemorrhages.
45
How to differentiate preseptal v. orbital cellulitis:
Both will have eyelid erythema and swelling, and chemosis.
Only orbital cellulitis will have pain w/EOM, proptosis +/- ophthalmoplegia w/diplopia.
Preseptal only need oral abx, orbital need IV abx +/- surgery.
46
How to differentiate Niemann-Pick from Tay-Sach:
Both have loss of motor milestones, hypotonia, feeding difficulties, and cherry-red macula.
Tay-sach: Hyperreflexia, no organomegaly
Niemann-pick: Areflexia, and hepatosplenomegaly
47
What tx might reduce risk of morbidity and mortality in Measles pts?
Vitamin A – given to all hospitalized Measles patients.
| It helps promote formation of Ab-producing cells and regeneration of epithelial cells
48
Tx of acute airway obstruction 2/2 mono:
IV corticosteroids
49
Potential ENT cxs of Mono:
Peritonsilar abscess, and acute airway obstruction
50
Findings of congenital syphilis:
Rhinorrhea, abnormal long-bone radiographs, enlarged placenta w/edematous umbilical cord and desquamating or bullous rash (can be on palms and soles), plus the features present in all congenital infections.
Most pts asx at birth and start displaying sxs 1-3mos
51
Clinical findings present in all the congenital (TORCH) infections:
Intrauterine growth restriction
Hepatosplenomegaly
Jaundice
Blueberry muffin spots
52
Features unique to congenital CMV infection:
Periventricular calcifications chorioretinitis, and microcephaly in severe cases
53
Features unique to congenital toxo infection:
Diffuse intracerebral calcifications
Severe chorioretinitis
Hydrocephalus
54
Features unique to congenital rubella infection:
Sensorineural hearing loss, cataracts and heart defects (PDA)
55
What causes the blueberry muffin spots in congenital infections?
Extramedullary hematopoiesis
56
What causes the IUGR in congenital infections?
Fetal inflammation
57
Features, sxs and tx of osteoid osteoma:
Benign bone-forming tm. most common at proximal femur.
Have pain worse at night, relieved by NSAIDs and unrelated to activity.
XR: small, round lucency
Tx: NSAIDs and monitor for spontaneous resolution
58
Pathophys of Intussusception 2/2 viral illness:
Hypertrophy of Peyer’s patches in the ileum serves as a nidus for telescoping.
59
Difference in tx for AOM and otitis externa:
AOM requires oral abx (amoxicillin)
| Otitis externa is tx’d w/ototopical abx
60
Most common causes of myocarditis in children:
Coxsackie B and Adenovirus
61
When should chelation therapy be used to tx lead poisoning?
When venous lead levels are >45 ug/dL
Dimercaptosuccinic acid (succimer) is used when levels are 45-69
Dimercaprol (British anti-Lewisite) + EDTA is used in emergency when levels are >70 or there are signs of acute encephalopathy.
62
How is bone age evaluation done?
Radiographic assessment of the hand and wrist to assess skeletal maturation.
63
When is the rotavirus vaccine given?
2-8 months only. If it is missed do not give past 8mo.
64
When is visual acuity testing performed?
Routinely at age 4. Can be performed as early as age 3 if the child is cooperative.
65
How to differentiate b/w Fe-deficiency anemia and thalassemia:
Fe-deficiency will have increased RDW, and low RBC count
Thalassemia will have Normal RDW, normal-high RBC count and likely high reticulocytes
Both will have microcytic cells. Fe-deficient will be hypochromic and Thals will have target cells.
Thals may also have elevated serum Fe and ferritin d/t increased RBC turnover.
66
When is varicella vaccine given?
2 doses – one at age 1 and 4 years
67
Postexposure pphx of varicella:
If completely immunized – nothing
Incompletely immunized and immunocompetent – VZV vaccine
Incompletely immunized and IMCPd – VZIG
68
What is laryngomalacia, its sxs, dx and mgmt?
It is increased laxity of supraglottic structures which causes inspiratory stridor worse when supine.
Peaks at 4-8mos.
Dx: normally clinical, confirmed w/flexible laryngoscopy for mod-severe cases
Mgmt: Reassurance (w/ spontaneous resolution by 18mo), or supraglottoplasty for severe sxs.
69
Cxs of SGA infants:
```
Hypoxia
Perinatal asphyxia
Meconium aspiration
Hypothermia
Hypoglycemia
Hypocalcemia
Polycythemia
```
70
Most common cause of congenital hypothyroidism:
Thyroid dysgenesis
71
Features of congenital varicella syndrome:
Limb hypoplasia, cataracts, and distinctive skin lesions (scarring). NO heart defects.
72
Most common risk factor for orbital cellulitis:
Sinusitis
73
Cause and findings of Beckwith-Wiedemann syndrome:
Dysregulation of imprinted gene in chromosome 11p15. Overproduction of IGF-2 – fetal hypoglycemia & hyperinsulinemia
Presents w:
Fetal macrosomia & rapid growth until late childhood
Omphalocele/umbilical hernia
Macroglossia
Hemihyperplasia (limbs larger on one side)
74
Cxs of Beckwith-Wiedemann and surveillance for them:
Wilms tm – abdominal US every 3 mo from birth to 8yr, then renal US from 8yr through adolescence.
Hepatoblastoma – Serum AFP every 3mo from birth to 4yr
75
Most common organisms and tx of septic arthritis in kids <3mo:
Staph, GBS, and GNR
Tx: Joint aspiration + abx
Abx – antistaph (nafcillin or vanc) + gentamicin or cefotaxime
(Blood and synovial fluid cultures should both be obtained before starting abx)
76
Most common organisms and tx of septic arthritis in kids >3mo:
Staph, GAS, and S. pneumo
Abx – Nafcillin, clindamycin, cefazolin, or vancomycin
**Blood and synovial fluid cultures should both be obtained before starting abx
77
Most common cause of osteomyelitis:
S. aureus
78
Perianal streptococcus features and tx:
Caused by S. pyogenes (GAS), common in school-age children.
Get bright, sharply demarcated erythema over perianal/perineal area. May have pruritis, pain w/stooling and fissures causing blood-streaked stool.
Tx w/oral abx (beta lactams – penicillin, amoxicillin)
79
Perianal dermatoses in infants:
Contact dermatitis #1 – spares creases/skin folds, tx w/barrier ointment or paste
Candida dermatitis #2 – beefy-red rash involving skinfolds w/satellite lesions. Tx w/topical antifungals
80
Most common predisposing factor of acute bacterial sinusitis:
Viral URI
| 1st line tx of bacterial sinusitis: amoxicillin + clavulonate
81
What is the pathogenesis of Minimal Change Disease?
T-cell mediated injury to podocytes causing increased molecular permeability to albumin.
82
Primary side effect of Hydroxyurea:
Myelosuppression
83
How to differentiate Marfan syndrome from Homocystinuria:
Both have pectus deformity, tall stature (increased arm:height and decreased upper:lower body), arachnodactyly, joint hyperlaxity, skin hyperelasticity and scoliosis
Marfan: AD, normal IQ, aortic root dilation, Upward lens dislocation
Homocystinuria: AR, decreased IQ, thrombosis, downward lens dislocation, megaloblastic anemia, fair complexion (blonde, blue eyes)
84
How to differentiate Ehlers-Danlos from Marfan/Homocystinuria:
All have joint laxity, skin hyperelasticity, but ED does NOT have tall stature, lens probs, or hypercoagulability.
ED does have scoliosis
85
What type of fluid is preferred for resuscitation in burn victims?
Lactated Ringers – it is considered a balanced solution and will help correct acidosis and maintain normal blood pH.
NS is an unbalanced solution and can cause hyperchloremic metabolic acidosis and hypocoagulability.
86
Do children w/febrile seizures need to be admitted for observation?
No. Only children with a post-ictal state who haven’t returned to baseline rapidly need admittance.
87
How to diagnose developmental dysplasia of the hip:
Age <4mos – hip US
| Age >4mos – hip XR
88
What does squatting do to help reduce cyanosis in ToF?
It increases systemic vascular resistance/afterload and decreases the R-to-L shunting across the VSD, which then increases flow across the RVOT.
89
How does the timing of pain differentiate b/w the differentials for non-traumatic joint swelling?
Infectious is an acute onset with constant pain.
Inflamm/Rheum is subacute/chronic with pain worse in the morning.
Neoplastic is subacute/chronic and has pain worse in the evening/night
90
Laboratory findings of the differentials for non-traumatic joint swelling:
Infectious – increased WBCs, platelets, and inflammatory markers
Inflamm/Rheum – Increased WBCs, platelets, and inflammatory markers. Decreased RBCs
Neoplastic – decreased WBCs and platelets
91
What is the common presentation of systemic-onset juvenile arthritis?
Chronic oligoarthritis (worse in the morning)
Daily fever (quotidian fever >2wks)
Rash (pink, macular, worsens during fever)
Possible lymphadenopathy and hepatosplenomegaly
92
Characteristic lab findings in sJIA:
Leukocytosis, thrombocytosis, and markedly increased inflammatory markers.
May also have anemia d/t chronic disease or Fe-deficiency
93
What should be investigated in someone w/subQ emphysema in anterior chest?
Pneumothorax – an emergent CXR should be obtained to rule it out.
SubQ emphysema often occurs 2/2 severe coughing paroxysms which can also lead to pneumothorax.
94
What is the tx of Croup/laryngotracheitis?
Mild (no stridor at rest): Humidified air +/- steroids.
Mod/Severe (stridor at rest): Steroids + nebulized racemic Epi
Shouldn't intubate/ventilate unless they have failed the above txs or have impending resp. failure (poor inspiratory effort, severe hypoxemia, etc.)
95
What are the initial steps in evaluating Primary amenorrhea?
Pelvic US to confirm presence of uterus.
If uterus is absent, do Karyotype.
If uterus is present then check FSH levels.
96
What are the differentials of Primary amenorrhea w/absent uterus on US?
Androgen Insensitivity
| Mullerian agenesis
97
What are the differentials of Primary amenorrhea w/present uterus on US and how are they differentiated?
Differentiate based on FSH levels:
Normal FSH – imperforate hymen
High FSH – Primary ovarian insufficiency (46, XX), Turner syndrome (45, XO)
Low FSH – Hypothyroidism, Prolactinoma, Functional HoThalamic amenorrhea (normal TSH, prolactin)
98
What is the mgmt. of scrotal hydrocele in a neonate?
Reassurance and observation up until 1 year. These are common and should resolve on their own by 1 year.
99
Most common cause of mastoiditis and its mgmt:
S. pneumo.
Complication of AOM.
Tx w/IV abx and drainage
100
What abx should be used to tx neonatal sepsis?
Ampicillin and Gentamicin
101
What is the acidified glycerol lysis test used to diagnose?
Aka the osmotic fragility test. Used to dx Hereditary Spherocytosis
102
What are the lab findings of Hereditary Spherocytosis(5)?
```
Increased MCHC
Spherocytes on peripheral smear
Negative Coombs test
Increased osmotic fragility on acidified glycerol lysis test
Abnormal eosin-5-maleimide binding test
```
103
How do pineal gland masses often present?
With Parinaud/Dorsal midbrain syndrome: limited upward gaze, upper eyelid retraction, pupils reactive to accommodation but not light.
Obstructive hydrocephalus: papilledema, HA, vomiting, and ataxia.
104
What are the common cxs of Bronciolitis?
Apnea (esp. infants <2mos)
Respiratory failure.
Risk of sepsis or bacterial pneumonia are extremely rare (<1%)
105
How do “flow” murmurs typically sound?
Grade I/II midsystolic ejection murmurs.
106
Most common cause of vaginal discharge in neonatal period?
Maternal withdrawal of estrogen. Presents w/bleeding as well. Lasts <1week.
107
Features of PKU:
AR mutation in phenylalanine hydroxylase.
Presents with severe intellectual disability, seizures, musty body odor, hypopigmentation of skin, hair, eyes and brain nuclei.
Dx via newborn screen or quantitative amino acid analysis (increased Phe)
108
What can provoke an absence seizure?
Hyperventilation
109
What comorbid conditions are often a/w Tourette syndrome?
ADHD and OCD
110
Most common cause of Sepsis in SCD?
S. pneumo – pts w/SCD should receive pphx penicillin until at least age 5 to avoid this.
111
Most common cause of Acute Bacterial Rhinosinusitis:
S. pneumo or non-typeable H. influenzae
112
Cxs of Bacterial meningitis in children:
~50% of children have long-term neurologic sequelae
Intellectual/behavioral disabilities
Hearing loss (2/2 inflammatory damage to the cochlea)
Cerebral palsy
Epilepsy
113
What type of virus is coxsackie?
An enterovirus
114
How is pertussis diagnosed?
Via pertussis culture or PCR. Will also see lymphocyte-predominant leukocytosis on CBC.
115
Most common congenital heart defect in patients w/Down syndrome:
Complete atrioventricular septal defect.
116
Most common cause of hearing impairment in children:
Conductive hearing loss d/t repeated ear infections.
117
Why are men with CF infertile?
Bc inspissated mucus collects and obstructs the developing vas deferens in utero and prevents them from forming.
118
What are the effects of untreated primary HoThyroidism on puberty?
Can cause peripheral/gonadotropin-dependent precocious puberty.
119
What are the features and management of Jervell Lange-Nielsen syndrome?
AR inheritance of molecular defects in K+ channels.
Features: long QT syndrome, and congenital sensorineural deafness.
Tx: BB (propranolol) + pacemaker if there’s hx of syncope.
Must also focus on maintaining normal levels of Ca2+, K+, and Mg2+
120
When should an infant be referred for audiology evaluation?
If they are not responding to their own name, babbling or saying small nonspecific words by 9mos.
121
Difference in presentation of sporadic and inheritable retinoblastoma:
Sporadic presents as unilateral disease.
| Hereditary is often bilateral
122
When is ceftriaxone used in Lyme disease?
IV ceftriaxone is reserved for Lyme meningitis and heart block (manifestations of early dissem’d Lyme)
123
Who can’t receive tetracyclines?
Kids <8 years and pregnant women.
124
Tx of Lyme in tetracycline contra’d pts:
Oral amoxicillin or cefuroxime (2nd-gen enteral Ceph)
125
What is Mupirocin?
Belongs to monoxycarbolic acid class of abx.
Has activity against most G+ cocci including MRSA and most strep (but not enterococci)
ONLY topical/intranasal agent w/MRSA coverage.
Used to tx impetigo and eradication of S. aureus nasal colonization
126
Alternatives to stimulants in ADHD tx:
Atomoxetine (selective NE-RI) is 1st line
| Others: a-2 adrenergic agonists (clonidine, guanfacine), Bupropion, TCAs
127
What type of exudative effusion will have increased TGs?
Chylothorax – often from disruption of the thoracic duct.
128
Transmission of Measles:
Airborne
129
Most common posterior fossa tumors in children:
```
#1 – Cerebellar astrocytoma (occur in lateral cerebellar hemispheres -- tremor, dysdiadokinesia etc.)
#2 – Medulloblastoma (occur in the cerebellar vermis -- truncal ataxia, gait instability)
```
130
What are the features of hemiplegic migraine?
Migraine HAs accompanied by motor weakness during the preceding aura phase.
Common in adolescents and typically do not cause LoC.
131
What is Todd paralysis?
Self-limited, focal weakness or paralysis that occurs after a focal or generalized seizure.
Presents in the post-ictal period w/hemiparesis or complete hemiplegia involving one side of the body.
132
What Rxs are used in the tx of Tourette?
First line are Antidopaminergic (Tetrabenzine – DoC, Risperidone, and Haloperidol).
A-2 adrenergic agonists (guanfacine, and clonidine) can be used as alternatives.
133
What is epidemic keratoconjunctivitis?
A more serious form of conjunctivitis caused by adenovirus.
Characterized by fever, eye pain and inflammation, and preauricular lymphadenopathy that’s followed by development of painful corneal opacities.
Self-limited, but highly contagious and occurs in outbreaks.
134
What disease is a/w 12/21 gene translocation?
Most common form of ALL
135
What is the definition of a concussion?
Transient alteration in consciousness following head trauma w/out intracranial imaging abnormality.
136
What are some features of Wiskott-Aldrich syndrome?
```
Eczema
Increased IgE and IgA, decreased IgM,
Micro-thrombocytopenia (small and low platelets) -- can get petechiae, purpura, hematuria, mucosal bleeding, intracranial hemorrhage
B and T cell dysfxn.
X-linked.
```
137
How is lice treated?
With permethrin lotion
138
What should all patients w/SLE be prescribed?
Hydroxychloroquine.
| It is a DMARD for SLE and improves survival and may be beneficial enough to let the patient stop taking steroids.
139
What do all patients with central precocious puberty need?
A brain MRI
140
Clinical Manifestations of Rickets (5):
Craniotabes (“ping-pong ball” skull)
Delayed fontanel closure
Enlarged: skull (frontal bossing) and long-bone joints (wrist widening)
Costochondral joint hypertrophy (“rachitic rosary”)
Genu Varum (femoral and tibial bowing)
141
Tx of radial head subluxation:
Hyperpronation of forearm or Supination of forearm w/flexion of elbow.
142
What may be an early sign of Rett syndrome?
Deceleration of head growth
143
What is Landau-Kleffner syndrome?
Deterioration/regression of language skills d/t severe epileptic attacks. Typically begins to deteriorate at age 3-6 years.
144
Features of Scarlet Fever:
Caused by S. pyogenes w/erythrogenic exotoxins
Fever & Pharyngitis w/ tonsillar exudates, palatal petechiae and strawberry tongue
Tender, shotty anterior cervical lymphadenopathy
Sandpaper rash most pronounced in skin folds – will desquamate and cause peeling of hands and feet.
Rash on cheeks may seem to cause circumoral pallor
Tx w/Penicillin (amoxicillin)
145
What are txs for nocturnal enuresis?
Enuresis alarm therapy and Desmopressin are 1st line
| Should not be implemented before age 5 as this is normal.
146
Features and tx of tinea capitis:
Dermatophyte infection most common in AfAm children.
Transmission: Direct contact or from fomite (sharing combs)
Clinical feats: Scaly, erythematous patch w/hair loss, +/- black dots in area, +/- tender lymphadenopathy (often occipital and post-auricular)
Tx: Oral griseofulvin or terbinafine
147
What are features of Congenital lymphedema and what is it commonly a/w?
Presents at birth with NON-pitting edema 2/2 lymphatic network dysgenesis – get protein-rich ISF in the hands, feet and neck.
Severe obstruction can result in cystic hygroma in the neck and fetal hydrops
Often a/w Turner Syndrome
148
What is the tx for Strawberry hemangioma?
Most require no tx.
Topical Propranolol is used for disfiguring, ulcerating, disabling (eyelid – strabismus), or life-threatening (those by airway)
149
What causes hyper-IgM syndrome?
X-linked defect in CD40-ligand on T-cells. Prevents class switching.
150
Tx of Immune Thrombocytopenia in children:
```
If only cutaneous sxs present – observation (norm resolves w/in 3mo).
If bleeding (decreased Hb/Hct, epistaxis, hematuria etc) – glucocorticoids, IVIG, or anti-D
```
151
Tx of Immune thrombocytopenia in adults:
If only cutaneous sxs and platelets >30K – observation
| If bleeding or platelets <30K – glucocorticoids, IVIG, or anti-D
152
Features of Neuroblastoma:
Neural crest origin, involves adrenal medulla and sympathetic chain.
Often presents <2yrs, w/abdominal mass. Can get Horner syndrome if in SNS chain
Other features: periorbital ecchymoses (orbital mets), spinal cord compression from epidural invasion, opsoclonus-myoclonus syndrome (rapid vertical and horizontal nystagmus w/myoclonus), HTN, flushing and diaphoresis
Dx: elevated CAs (VMA, HVA), n-myc amplification, small round blue cells on histo.
153
How should height and weight change in an infant by 12mos?
Weight should triple and height should increase by 50% from birth weight and height.
154
Most common causes and risk factors of Otitis Externa:
P. aeruginosa and S. aureus
Risk Factors: Water exposure, Trauma (cotton swab), Foreign material (hearing aid, headphones), derm conditions (eczema, contact dermatitis)
155
Tx of Otitis Externa:
Remove debris, topical abx (fluoroquinolone), +/- topical glucocorticoid
156
What CNS cxs are NF1 patients most at risk of developing?
Optic pathway gliomas
Astrocytomas
Brainstem gliomas
The risk carries into adulthood
157
PE findings in Von Gierke disease:
Doll-like face w/rounded cheeks, thin extremities, short stature, and protuberant abdomen d/t hepatomegaly.
***Spleen and heart are not involved***
158
Laboratory findings in Von Gierke (Type I glycogen storage) disease:
Presents around 3-4 mo w/glycogen accumulation, hypoglycemia (causes seizures), lactic acidosis, hyperuricemia, and hyperlipidemia.
159
Enzyme deficient in Von Gierke:
Glucose-6-phosphatase
160
Enzyme deficient in Gaucher disease:
Glucocerebrosidase
161
What causes Wiskott-Aldrich?
XR defect in WAS protein gene – impaired cytoskeleton changes in leukocytes and platelets.
The actin cytoskeleton in WBCs in abnormal, so there’s immune dysfxn d/t impaired cellular migration and immune synapse formation.
Get bacterial, viral and fungal infections.
162
What is Werdnig-Hoffman syndrome?
An AR disorder – degeneration of anterior horn cells & cranial nerve motor nuclei causes “floppy baby” syndrome.
163
Risk factors for neonatal respiratory distress syndrome:
```
#1: Prematurity
Others: Male sex, perinatal asphyxia, maternal DM, c-section.
```
164
What are some factors that might decrease the risk of neonatal RDS?
IUGR, maternal HTN, and prolonged rupture of membranes.
| ie: any type of intrauterine stress
165
Features of Leukocyte adhesion deficiency:
Delayed umbilical cord separation (>3 weeks), recurrent skin and mucosal bacterial infections (w/out purulence), and severe periodontal disease.
CBC shows marked leukocytosis w/NP predominance.
166
What should be suspected in a pt. w/continued ear drainage for several wks. despite abx tx?
Cholesteatoma
167
Common causes of pediatric stroke:
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Sickle Cell
Prethrombotic disorders
Congenital cardiac disease
Bacterial meningitis
Vasculitis
Focal cerebral arteriopathy
Head/neck trauma
```
168
What causes the primary insult in refeeding syndrome and what are the sxs?
Rapid increase in insulin
| Get arrhythmia, CHF, seizures, and Wernicke encephalopathy.
169
What is the CoD in Friedreich ataxia?
Cardiac dysfxn by 30-40 yrs
| Arrhythmia, CHF etc.
170
Common presentation of Friedreich ataxia:
Progressive gait ataxia, dysarthria, loss of vibration/proprioception, absent DTRs, HOCM, scoliosis and DM.
Presents in adolescence/early adulthood
171
Features of ataxia-telangiectasia:
AR disorder of DNA repair – have immunodeficiency, progressive cerebellar ataxia, ocular and cutaneous telangiectasias and increased risk of malignancy.
172
What are the likely HbF levels to be in an SCD patient on Hydroxyurea?
>15%
173
Features of Sturge-Weber syndrome:
Congenital NON-inherited anomaly of neural crest derivatives d/t activating mutation of GNAQ
Port wine stain in V1/V2 distribution (nevus flammeus)
Leptomeningeal capillary-venous malformation
Seizures +/- hemiparesis
Intellectual disability
Visual field defects
Glaucoma
174
What is Kippel-Trenaunay syndrome?
A syndrome of capillary, venous or lymphatic malformation in combination w/limb overgrowth.
They have port wine stains on the lower extremities
No neuro defects/abnormalities
175
Where are ependymomas most likely located?
In children most common site is the 4th ventricle in the posterior fossa.
They arise from ependymal cells lining the ventricles and spinal cord.
176
What are the most common Supratentorial CNS tms in children?
Pilocytic astrocytoma > Craniopharyngioma > Glioblastoma.
177
Most common cx of Mumps?
Aseptic meningitis – get HA, fever and nuchal rigidity.
| In post-pubertal males orchitis may also occur and cause impaired fertility.
178
How does Mumps present?
Fever and parotitis – facial swelling with tenderness.
| Swelling often obscures the angle of the mandible.
179
Features of Craniopharyngiomas:
Calcified, intracranial tumors in the suprasellar region.
| Present w/bitemporal hemianopsia and pituitary hormonal deficiencies (DI, GH deficiency)
180
What is the DoC for hemodynamically stable pts w/heavy vaginal bleeding?
High-dose combined OCs or IV estrogen.
| These stabilize the endometrium and stop acute bleeding.
181
What are Brushfield spots?
Whitish-grey spots on the periphery of the iris – often present in Down syndrome pts.
182
What are some features of von Hippel-Lindau syndrome?
Inherited disorder – causes both benign and malignant tumors.
Benign hemangioblastomas (high vascularity w/hyperchromatic nuclei) occur in the retina, brain stem, cerebellum and spine.
Angiomatosis – cavernous hemangiomas in skin, mucosa and organs (even CNS – seizures)
Bilateral RCCa
Pheochromocytomas
183
Disinhibited social engagement disorder:
Overfamiliarity and an unhesitant approach to unfamiliar adults.
Often seen in children of early neglect.
184
Reactive attachment disorder:
Often develops in young children of abuse, neglect, institutionalization or inconsistent care.
The children rarely seek comfort and don’t respond to attempts to comfort them, have lack of social response, lack of positive emotions, and episodes of unexpected irritability or sadness.
185
What would an EKG in tricuspid atresia show?
L axis deviation (normal neonates have R axis deviation)
Small/absent R waves in precordial leads (V1-V3)
Tall, peaked P waves (bc increased flow into RA from ASD)
186
Features of tricuspid atresia:
Lack of tricuspid valve – requires ASD or VSD for survival.
Lack of blood flow to RV and pulmonary outflow tract – hypoplastic R heart and underdevelopment of pulmonary valve and/or a.
Decreased pulm circulation – decreased pulm markings on CXR.
187
Mgmt of vaginal foreign bodies in chdlren:
Warm irrigation and vaginoscopy under sedation/anesthesia
188
What is the most common cause of Pneumonia in CF children v. adults?
In children, S. aureus is the most common and Pseudomonas is in adults.
S. aureus starts to decline around age 20 whereas pseudomonas begins to peak.
Both pathogens should be covered in tx of pneumonia in CF children.
189
What renal disease are HBV+ people most at risk of getting?
Membranous nephropathy.
| Normally uncommon in children, but can occur in those w/HBV
190
What cyanotic heart diseases of the newborn present with a single S2?
Transposition – Single S2 +/- VSD murmur
Tricuspid atresia – Single S2 + VSD murmur
Truncus arteriosus – Single S2 + systolic ejection murmur
191
Most common congenital cyanotic heart disease in the neonatal period:
Transposition of the great vessels.
192
Tx of Ringworm (Tinea Corporis):
1st line/localized disease - -topical clotrimazole or terbinafine
2nd line/extensive disease – oral terbinafine or griseofulvin
193
What are the features of Diamond-Blackfan anemia?
Disease of congenital erythroid aplasia.
Clinical: Craniofacial abnormalities (webbed neck, cleft palate, short stature), triphalangeal thumbs, and increased risk of malignancy
a/w polyhydramnios on prenatal US
Lab: Macrocytic anemia, reticulocytopenia, normal platelets and WBCs.
Tx: steroids and RBC transfusions
194
What kind of anemia is Diamond-Blackfan?
Pure red cell aplasia, macrocytic.
Caused by defect in RBC progenitor cells that increases RBC apoptosis and causes profound anemia
Will also see reticulocytopenia, but will have normal platelets and WBCs
195
What organisms likely cause pseudoappendicitis and how are they differentiated?
Y. enterocolitica – also has erythema nodosum (1-2cm red nodules tender to palpation)
C. jejuni – no erythema nodosum
196
What additional vaccines should HbSS children receive at age 2?
PPSV 23 (should have already received PCV13)
MCV4 (meningococcal)
Also influenza if they haven’t had it yet that year
197
When should pap smears be recommended?
Only age 21 and later – then to all women regardless of sexual hx
198
Tx of Kawasaki:
IVIG and HIGH-dose aspirin
199
Why do Kwashiorkor children have large bellies?
Ascites
Dilated loops of bowel
Enlarged, fatty liver – d/t decreased apolipoprotein synth and decreased fat exportation
200
What kind of organism is Listeria?
Gram + rod
201
What is West Syndrome and how is it treated?
A pediatric seizure disorder characterized by the triad of:
Infantile spasms
Developmental regression
Hypsarrhythmia on EEG – pathognomonic, disorganized background w/high-A waves and spikes. Seen interictally as well.
Tx: ACTH (DoC). Vigabatrin may be considered as an alternative
202
What test should be done repeatedly in pts. w/Kawasaki?
Echo
203
What is the most specific sx that would suggest leukemia in the setting of pancytopenia?
Bone Pain
204
How do infants w/urea cycle defects typically present?
W/in 24-48hrs with poor feeding, tachypnea, and lethargy.
Hyperammonemia will cause central hyperventilation leading to Respiratory alkalosis.
CBC, lactate, electrolytes, glucose and ketones will all likely be normal.
205
How to differentiate pyruvate metabolism disorders from urea cycle defects:
Both will have hyperammonemia, but Pyruvate will also have lactic acidemia.
206
What will be seen on CXR in an infant w/meconium aspiration?
Coarse, irregular infiltrates and hyperexpansion.
207
What should be expected in a patient who has an acute decompensation on mechanical ventilation?
Pneumothorax.
208
What is the major life-threatening cx of ALL?
Infection – so in any child w/fever in the setting of suspected ALL abx should be started asap before confirming diagnosis.
209
What valve abnormalities does Acute rheumatic fever cause?
FIRST mitral regurg
| Then years later the valve may become stenotic.
210
What is the best tx of Von Gierke disease?
Uncooked cornstarch – it is a glucose polymer.
211
Why isn’t ceftriaxone used in infants?
It increases the risk of hyperbilirubinemia by binding to albumin and displacing the BR.
212
Characteristic finding on AXR of NEC:
Pneumatosis intestinalis – air in the bowel wall.
213
What is the most sensitive and specific test for iron-deficiency anemia?
Serum ferritin levels.
| Should order these over Fe levels.
214
What is the best way to asses urine protein excretion?
To determine the mg of protein/mg of Creatinine ratio.
This is easier than measuring 24hr proteins and such.
Nephrotic range proteinuria is considered >3mg protein/mg creatinine on a spot urine sample.
215
How will classic galactosemia present and what will be seen on labs?
Within days-weeks after introduction of formula/breastmilk babies develop lethargy, poor feeding, jaundice, cataracts, and metabolic acidosis.
Labs: direct hyperbilirubinemia, elevated transaminases, prolonged PT and aPTT, hypoglycemia and aminoaciduria.
216
First line tx of thrush:
Topical Nystatin
| Fluconazole can be used in cases unresponsive to Nystatin
217
What disease is characterized by a defective production of neutrophil superoxide?
Chronic Granulomatous disease – defect in phagocyte NADPH oxidase.
218
What are significant developmental milestones seen at 18mos?
Walk backwards, run, walk up steps, scribble, build a tower of 2 cubes, point to at least one body part, say six words, remove garments.
This is the first age of autism screening, and then second is at 24 mos.
219
When should autism screening be done?
18 and 24 mos
220
When should hearing and vision screening be done?
Vision – 3 and 4 years old
| Hearing – 4 years old
221
When does BP become a routine part of the physical exam?
After age 3yr
222
When should lead screening be done?
At age 1 and 2 years.
223
What ocular abnormalities are seen in Alport syndrome?
Lens dislocation, posterior cataracts, and corneal dystrophy
224
Features of Fanconi Anemia:
Inherited DNA repair defect – bone marrow failure
Clinical: short stature, hypo-/hyperpigmented macules, abnormal (hypoplastic or absent) thumbs, and genitourinary malformations
Labs: pancytopenia, + chromosomal breakage testing.
225
What type of anemia is typically seen in Fanconi anemia?
Macrocytic anemia and pancytopenia d/t bone marrow failure
226
What type of shunt does a PDA cause?
An extracardiac L-to-R shunt aka it shunts blood from the systemic to pulmonary circulation.
227
What organism causes tinea capitis?
Most commonly in the US – Trichophyton tonsurans
228
What procedure should be done to tx severe (stage 4) GERD?
Fundoplication
229
What Rx would be best to give a patient with signs of Cardiogenic shock w/decreased renal fxn?
Dopamine.
230
Possible complication of topical anesthetic use?
Methemoglobinemia.
| Dapsone and nitrites may cause this as well.
231
What are helmet cells?
Schistocytes
232
What are Howell-Jolly bodies?
Normal nuclear remnants of RBCs – normally removed by the spleen, so seen in ppl w/hx of splenectomy or functional asplenia (SCD).
233
How would SLE likely present in childhood?
With neuropsychiatric sxs, immune-mediated anemia (Coombs +), thrombocytopenia, arthritis, and nephritis
234
What screening exams should be performed regularly on children with JIA?
Slit lamp exams to check for ocular involvement – anterior uveitis etc.
235
What is physiologic jaundice?
Unconjugated hyperbilirubinemia that occurs >24hrs (norm about 72hrs) and goes away by one week.
D/t immature liver conjugation enzymes.
ALL JAUNDICE <24hrs IS PATHOLOGICAL
236
What kind of medication is Clotrimazole?
Topical anti-fungal
237
What kind of medication is Triamcinolone?
Topical corticosteroid
238
What Immunodeficiency disorder may present after administration of BCG vaccine?
IL-12 receptor deficiency – get disseminated mycobacterial and fungal infections, and decreased IFN-y
239
What Immunodeficiency disorder has increased risk of gastric ca?
CVID – also increased risk of lymphoma.
240
What are the primary disorders of B-cells only?
XLA – bacterial and enteroviral infections. Absent B cells in peripheral blood.
IgA deficiency – Airway and GI infections. A/w atopy, AI, and anaphylaxis. Only IgA decreased
CVID – defect in B-cell differentiation. Increased risk of AI dxs, bronchiectasis, lymphoma, gastric ca., and sinopulmonary infections. Decreased plasma cells and Igs. Presents later.
241
Primary disorders of T-cells only:
DiGeorge
IL-12R deficiency
Hyper-IgE aka Job syndrome
Chronic mucocutaneous candidiasis
242
Immunodeficiencies of B&T cell dysfxn:
SCID
Ataxia-telangiectasia
Hyper-IgM syndrome
Wiskott-Aldrich syndrome
243
Immunodeficiences of phagocyte dysfxn:
LAD
Chediak-Higashi
Chronic granulomatous disease
244
Most common type of SCID:
IL-2R gamma chain defect – X-linked
| 2nd – ADA deficiency, AR
