Pediatrics Flashcards
1
Q
Albuterol
A
Short-acting β2-adrenergic receptor agonist used for the relief of bronchospasm in conditions such as asthma and chronic obstructive pulmonary disease. It is marketed as Ventolin among other brand names.
2
Q
Bulimia nervosa PE finding
A
enlarged parotid gland
3
Q
Coombs Test
A
Test for autoimmune hemolytic anemia (is used to detect if antibodies or complement system factors have bound to RBC surface antigens in vivo)
4
Q
Prevnar
A
Pneumococcal vaccine used to protect infants and young children against disease caused by the bacteria Streptococcus pneumoniae (pneumococcus).
5
Q
Palivizumab/Synagis
A
monoclonal antibody for RSV
6
Q
Tacrolimus
A
Immunosuppressive (IL2 antagonist) drug that is mainly used after allogeneic organ transplant to reduce the activity of the patient’s immune system and so lower the risk of organ rejection. Also treats atopic dermatitis (a skin rash and type of eczema).
7
Q
Nystatin
A
An antifungal medication to which many molds and yeast infections are sensitive, including Candida.
8
Q
Germinal matrix
A
A fragile portion of the brain that may be damaged leading to an intracranial hemorrhage, which is the most common cause of second trimester spontaneous abortions.
9
Q
What are the TORCH infections?
A
Toxoplasmosis Other (syphilis) Rubella Cytomegalovirus (CMV) Herpes simplex virus (HSV)
10
Q
5 “T”s of Cyanotic Congenital Heart Disease
A
- Tetralogy of Fallot (TOF)
- Transposition of the Great Arteries (TGA)
- Truncus Arteriosus
- Tricuspid Atresia
- Total Anomalous Pulmonary Venous Connection (TAPVC)
11
Q
Normal Bilirubin Levels
A
Total 0.2-1.0
Conj. 0-0.2
12
Q
Highest Risk Factor of Cerebral Palsy
A
prematurity (78% of CP pts)
13
Q
Doxycycline treats…
A
IN CHILDREN OVER 8, it treats Lyme disease, chronic prostatitis, sinusitis, pelvic inflammatory disease, acne, rosacea, and rickettsial infections. It is contraindicated in children under 8 and pregnant women because it can cause permanent teeth stains.
14
Q
Ewing’s Sarcoma
A
Highly malignant tumor found most often in the lower extremity, at the metaphysis and diaphysis of the femur, then tibia and humerus. Typically occurs in white males 0-20 yo. Has on “onion skin/lamellated” appearance.
15
Q
Osteomyelitis
A
Presents with fever, joint pain, and swelling. Shows a central lytic bone defect with surrounding sclerosis.
16
Q
Nursemaid’s Elbow
A
Also called subluxed radial head. Common elbow injury for 1-5 yo children, occurs when someone pulls upwards on their arm. They will present with pronated elbow and will refuse to move it.
17
Q
Guillain-Barre Syndrome
A
An ascending polyneuropathy that usually presents one week after viral infections (like campylobacter jejuni!!!!) involving peripheral motor nerves, and sometimes sensory and autonomic nerves. Symptoms appear from the feet up and disappear in reverse order.
Treat with IVIG and steroids.
18
Q
Scarlet Fever
A
Features erythematous SANDPAPER rash on neck, axillae, & groin initially, then spreads (BEGINS ON TRUNK). Blanchable. See Pastia’s Lines of petichiae. Desquamation of skin as rash resolves.
Fever, chills, tender lymphadenopathy.
Pharynx is red and covered in grey-white exudates. Desquamation occurs after a week. Caused by GABHS endotoxin, can follow strep pharyngitis (!!!) or skin infection. Treat with Penicillin or erythromycin.
Sequelae include: rheumatic fever and PANDAS/OCD/tics (preventable), post-strep GN and post-strep arthritis (nonpreventable).
19
Q
Staphylococcal scalded skin syndrome
A
Skin condition characterized by superficial flaccid bullae followed by extensive exfoliation of skin. Common in infancy, rare after 5yo.
20
Q
Herpangina
A
Throat infection caused by enteroviruses, especially Coxsackievirus, characterized by high fever and severe sore through that may result in an inability to swallow. Ulcerative lesions may be found on palate, tonsils, pharynx. Supportive treatment.
21
Q
Most common causative organisms of otitis media:
A
Strep. pneumoniae (40%)
H. flu (25-30%)
Moraxella catarrhalis (10-15%)
About 30% caused by viruses (RSV & rhinovirus)
The rest (~5%) caused by S. aureus, gram-negative bacteria, Group A strep, found mostly in neonates.
Treatment: 10 days of amoxicillin.
22
Q
Beckwith-Wiedmann Syndrome
A
Overgrowth Disorder
Path: Disregulation of imprinted gene expression in ch 11p15 (IGF2, insulin-like GF 2)
PE: Fetal macrosomia, rapid growth until late childhood, umbilical hernia, macroglossia, hemihyperplasia
Complications: Wilms Tumor, hepatoblastoma
Surveillance: serum a-fetoprotein, abdominal/renal ultrasound, monitor hypoglycemia in newborns only.
23
Q
Infectious Mononucleosis
A
Most likely caused by EBV.
24
Q
Meningitis neck restriction
A
Associated with flexion more than extension.
25
Normal total leukocyte count
4,500-11000 cells/mm3
26
Encapsulated bacteria
```
Haemophilus influenzae type b (Hib)
Streptococcus pneumoniae (pneumococcus)
Neisseria meningitidis (meningococcus)
Group B streptococcus (GBS)
Klebsiella pneumoniae
Salmonella typhi
```
A popular mneumonic to remember most of the encapsulated bacteria is the SHiNS bacteria (S. pneumo, HiB, N. meningitidis, Salmonella).
27
Pediatric Strokes
Uncommon, so usually caused by a congenital abnormality, infection, or systemic illness. One of the most common causes is sickle cell anemia.
28
What does a measurement of serum antineutrophil cytoplasmic antibodies evaluate?
It evaluates for the presence of a vasculitis, a potential cause of stroke in children and young adults.
29
Lennox–Gastaut syndrome (LGS), also known as Lennox syndrome
A difficult-to-treat childhood-onset epilepsy .
```
appears between 2-6 yo
frequent seizures
different seizure types
developmental delay
psychological and behavioral problems
```
30
Niacin/B3 Deficiency
Deficiency of this causes pellagra, which is characterized by diarrhea, dermatitis (painful and looks like a sunburn), and dementia, as well as “Casal's necklace” lesions on the lower neck, hyperpigmentation, thickening of the skin, inflammation of the mouth and tongue, digestive disturbances, amnesia, delirium, and eventually death, if left untreated. Common psychiatric symptoms include irritability, poor concentration, anxiety, fatigue, restlessness, apathy, and depression.
See in people with bowel disease or in cultures where they eat a lot of corn.
31
Thiamine/B1 Deficiency
beriberi/Wernike-Korsakoff Syndrome, characterized by neuro and psych symptoms, seen in alcoholics or patients who've had weight loss surgery.
32
Riboflavin/B2 Deficiency
Can lead to cheilosis, glossitis, seborrheic dermatitis (often affecting genital areas), pharyngitis, and edema/erythema of the mouth.
33
Cyanocobalamin/B12 Deficiency
Causes macrocytic anemia and peripheral neuropathy.
34
Normal Leukocyte Counts
The reference range for adults (males and females) is as follows:
```
Total leukocytes: 4.00-11.0 x 109/L
Neutrophils: 2.5–7.5 x 109/L
Lymphocytes: 1.5–3.5 x 109/L
Monocytes: 0.2–0.8 x 109/L
Eosinophils: 0.04-0.4 x 109/L
Basophils: 0.01-0.1 x 109/L
```
35
Intususception
This GI disease may present in an infant or a young child with EPISODIC abdominal pain (pull legs up to chest), irritability, vomiting, red currant jelly stools, and an abdominal mass. Diagnose with a air or barium enema.
See "target sign" on ultrasound.
Common in kids with HSP.
36
Meckel's Diverticulum
Typically presents with PAINLESS melena/tarry/maroon stools in 2-3 yo children. It is the most common anomaly of the GIT and it results from the failure of the vitelline duct to obliterate during fetal development. Gastric tissue may be present, which results in ulcerations and bleeding. Diagnosis made with technetium-99m pertechnetate scanning (uptake by heterotropic gastric mucosa).
37
Normal IQ Range
Mean for the population: 100.
2 SD below the mean: 70
Intellectual Disability:
Mild – from between 50 and 55 to approximately 70
Moderate – from between 35 and 40 to between 50 and 55
Severe – from between 20 and 25 to between 35 and 40
Profound – from <20 to 25
38
Fragile X Syndrome
Increased number of CGG trinucleotide repeats, mutation in the FMR1 gene. Characteristic appearance: large head, long face, prominent forehead and chin, large ears, large testicles.
Behavior: Hyperactivity, autism, temper tantrums. Most commonn inherited form of mental retardation.
Low to normal IQ, language disability.
39
Fetal Alcohol Syndrome
Mild to moderate mental retardation, hypo-plastic maxilla, long philtrum, microcephaly.
40
Reye Syndrome
A rare illness seen exclusively in children less than 15 yo who were treated with salicyclates/aspirin for a viral infection. Present with vomiting, agitation, irrational behavior, lethargy, stupor, restlessness, possibly convulsions.
ASPIRIN IN CONTRAINDICATED IN YOUNG CHILDREN WITH VIRAL INFECTIONS.
Labs: hyperammonia, slightly elevated bili and alk phos, prolonged PT time, hypoglycemia, and moderate to severe elevations in ALT, AST, and lactate dehyd. levels.
Biopsy of liver, kidneys and brain reveals microvesicular steatosis. Treatment is supportive.
41
Systemic Carnitine Deficiency (SCD)
Inherited defect in FA oxidation. Clinical features: hypoglycemia, hypoprothrombinemia, acute episodes of encephalopathy.
42
Clubbed Fingers
CF!
43
Gram Positive Diplococci
Streptococcus pneumoniae
44
Gram Positive Cocci
Staphylococcus spp.
45
Gram Negative Cocci
Neisseria
46
Gram Positive Rods
Listeria, Bacillus cereus and anthracis
47
Gram Negative Rods
Pseudomonas, Haemophilus, Klebsiella, Legionella
48
CMV pneumonia
Pneumonia that occurs in immunocompromised pts with CD4<50.
49
Pneumonia in immunocompromised hosts with CD4<200
Pneumocystis jiroveci, Cryptococcus neoformans, Histoplasma capsulatum, CMV (CD4<50)
50
3 most common causative organisms in CF pneumonia
Haemophilus influenza
Pseudomonas a.
Staphylococcus
51
Fancy word for "a scratch"
excoriation
52
Congenital CMV or Toxo Infection S&Sx
Developmental delay, IUGR (including microcephaly), cataracts, seizures!!!, HSM, prolonged neonatal jaundice, and purpura at birth. Calcified brain densities in periventricular area (CMV) or diffusely throughout cortex (Toxo)
53
Down Syndrome/Trisomy 21 features
Endocardial cushion defect or ASD, hypotonia with poor Moro reflex, flat face, slanted palpebral fissures, laxity of joints/flexible (esp. atlantoaxial instability), excessive skin on the back of neck, cardiac defects, duodenal atresia, extra space between first two toes
54
Edward's Syndrome/Trisomy 18 features
```
Features:
Clenched hand, with overlap
Intrauterine growth retardation (IUGR)
Rocker bottom feet
Micrognathia, prominent occipital, micro-ophthalmia
Low set ears
Cardiac defects
```
More: Weak cry, single umbilical artery, SGA, low-set malformed ears, microcephaly, rocker-bottom feet, inguinal hernias, cleft lip/palate, micrognathia. Also clenched hands with overlapping digits, small palpebral fissures, prominent occiput, short sternum, cardiac defects (VSD, ASD, PDA, or aortic coarctation).
55
Patau Syndrome/Trisomy 13 features
```
Features:
Holoprosencephaly
Polydactyly
Microcephaly
Midline Cleft Lip/palate
Cutis aplasia ("punched out lesions" of the scalp)
```
More: SGA, dysmorphic, microcephaly, sloping forehead, deafness, cutis aplasia, polydactyly micropthalmia, omphalocele. Also holoprosencephaly, cleft lip, polydactyly, overlapping fingers, coloboma, omphalocele, and cardiac defects (VSD, ASD, PDA, dextrocardia).
56
Conditions associated with asthma
GERD, eczema, sleep-disordered breathing, allergic rhinitis, sinusitis
57
albuterol
Short-acting beta-2 agonist (bronchodilator)
58
Low birth weight (LBW)
birth weight < 2,500 g (5 lbs 8 oz)
59
Very low birth weight (VLBW)
birth weight < 1,500 g (3 lbs 5 oz)
60
Conditions associated with prematurity
hypoglycemia, intraventricular hemorrhage, respiratory distress syndrome, pneumonia,h transient tachypnea of the newborn, patent ductus arteriosus, persistent fetal circulation, necrotizing enterocolitis, hyperbilirubinemia, kernicterus, cerebral palsy
61
Conditions associated with high dose O2 given to neonates
pulmonary fibrosis, bronchopulmonary dysplasia, retrolental fibroplasia
62
Henoch Schonlein Purpura
A systemic vasculitis characterized by deposition of IgA immune complexes, exact etiology unknown.
Often preceded by URI
Palpable purpura on lower extremities and butt
joint and abdominal pain (***Can develop intussusception or GI hemorrhage!!**)
renal disease (IgA deposition)
SCROTAL PAIN and SWELLING
Normal platelet count. Treatment is supportive.
63
technetium-99m pertechnetate
Uptake by heterotropic gastric mucosa to diagnose Meckel's Diverticulum
64
Continuous flow murmur
Patent Ductus Arteriosus (high pressure aorta to low pressure pulm artery). Sounds like machinery.
65
Klumpke Palsy
"Claw hand": Extended wrist, hyperextended MCP joints, flexed prox and distal interphalangial joints, absent grasp reflex. Also miosis and ptosis (Horner Syndrome).
Due to shoulder dystocia and subsequent traction of C8 and T1 nerves during delivery. Risks: LGA, maternal diabetes, maternal obesity.
66
Erb's Palsy/Duchenne Palsy
Injury to C5-C6
Decreased moro and biceps reflexes on affected side, "Waiter's tip" (extended elbow, pronated forearm, flexed wrist and finger), INTACT grasp reflex.
67
Enuresis treatment medication
DDAVP (desmopressin, a vasopressin mimetic) or imipramine
68
Haloperidol uses
Tourette syndrome
| Schizophrenia
69
Clonidine drug use
Centrally acting alpha-agonist that is used to control blood pressure.
70
Friedreich Ataxia
Autosomal recessive condition characterized by excessive trinucleotide repeats resulting in an abnormal tocopherol transfer protein. Degeneration of nerve tissue in the spinal cord, in particular sensory neurons essential (through connections with the cerebellum) for directing muscle movement of the arms and legs.
Progressive disease with poor prognosis. Death at 30-35 years old. 90% develop myocarditis and die of this.
Parents need genetic counseling.
71
When are kids able to copy a cross, circle, square, rectangle, triangle and a diamond?
3 years: cross, circle
4 years: square, rectangle
5 years: triangle
6 years: diamond
72
Most common peds osteomyelitis causing organisms
Staphylococcus aureus (most common)
Others are GBS and E coli in infants, Strep pyo in children.
73
Osteomyelitis in kids with sickle cell is usually caused by...
Salmonella
74
Cervical Lymphadenitis etiologies
1. Bacterial infection (S. aureus, S. pyogenes, TB, Myco. avium, B. henselae)
2. Reactive lymphadenitis
3. Viral Infections (EBV, CMV, HIV)
4. Kawasaki Disease
5. T. gondii
6. Structural lesions on neck
75
Kawasaki Disease
An acute febrile illness of childhood or unknown origin. Most common cause of acquired heart disease in children in the US. M:F 3:2, most common in Asians, mean age is 18-24 mo.
Features: fever >5d, bilateral conjunctivitis with limbic sparing (no exudate), pharyngitis, strawberry tongue, red cracked swollen lips, unilateral cervical lymphadenitis, rash on trunk, edema of the hands and feet, (later) peeling around nailbeds.
Concern for coronary artery aneurisms (20%).
Labs: high ESR and CRP. LFTs can be elevated.
Treatmt: IVIG & aspirin within 10 days for start of fever.
76
T. gondii Infection
Intracellular parasite transmitted through direct contact with cat feces, undercooked meats, fruits or veggies, transplacental passage. Causes a mononucleosis like illness with cervical lymphadenopathy (malaise, fever, sore throat, myalgias, lymphadenopathy, rash, HSM).
Diagnose with antibody titers against protozoa.
Important pathogen of immunosuppressed people, like in HIV. Can present with focal seizures. Treat only immunocompromised, pregnant women, and newborns. Sulfadiazine and pyrimethamine.
77
Bartonella henselae
"Cat scratch disease" is caused by the gram-negative B. henselae.
Regional lympadenopathy (axillary, cervical or inguinal) after a cat/kitten bite/scratch. Results in a papule along the scratch and lympadenopathy 1-2 weeks later. Lymphnode is red, warm, and tender.
Diagnose with IgM ab. to B henselae. Treat with supportive care.
78
Parotitis (swollen parotid salivary gland) causes
1. Mumps virus & other viruses (CMV, EBV, HIV, influenza) - bilateral involvement
2. Bacterial infection (Staph. aureus, Strep. pyo/GABHS, TB). Uncommon in children. UNILATERAL involvement
79
Post-streptococcal Glomerulonephritis
Present with hypertension and cola-colored urine.
Seen after streptococcal pharyngitis, impetigo/SSSS, Scarlet fever
Antibiotic therapy DOES NOT prevent this complication.
80
PANDAS (Ped. Autoimmune Neuropsychiatric Disorders associated with Streptococcal infection)
Phenomenon in which patients develop the acute onset of OCD symptoms or a tic disorder after strep infection. Antibiotic therapy DOES prevent this complication.
Treat PANDAs with OCD therapy--Fluoxetine SSRI (Prozac)
81
IVIG
A blood product administered intravenously. It contains the pooled, polyvalent, IgG antibodies extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months.
```
Used to treat:
Allogeneic bone marrow transplant
Chronic lymphocytic leukemia
Many primary immunodeficiencies
Idiopathic thrombocytopenic purpura
Pediatric HIV
Kawasaki disease
Chronic inflammatory demyelinating polyneuropathy (CIDP)
```
82
Differential for bloody stools in a 1 month old:
```
Infectious colitis
Diaper rash or anal fissure
Sign of sepsis/infection
Bleeding disorder
Milk protein intolerance
```
83
Mid-Parental Height
Boys: [(Mom's H + 13 cm) + Dad's Height] /2
Girls: [(Dad's H - 13 cm) + Mom Height] /2
13 cm = 5 in
84
How many calories in 1 oz of formula?
20 kcal
85
Still's Murmur
Physiologic murmur heard in 30-50% of normal children aged 3-8 yo. Should disappear at puberty.
86
Peripheral Pulmonic Stenosis
Physiologic: turbulence in the pulmonic vasculature in newborns. Should disappear by 6-12 months of age. Murmur is louder in the back and axillae than in the precordium.
Pathologic: Can be caused by Williams syndrome (supravalvular aortic stenosis) or Alagille syndrome
87
Meningitis in a 6 year old: likely orgs, treatment
Orgs: pneumococcus, meningococcus (N. meningitides)
Abx: vancomycin, cefotaxime (3rd gen cef)
88
Pathogens causing URI then arthritis
S. aureus, Group A Strep, Neisseria if sexually active.
Start vanc or clinda
89
Another name for Strep pyogenes
Group A Strep, GABHS
90
Turner syndrome
45X. Appearance: Short stature, widely spaced nipples, webbed neck/excessive nuchal skin, swollen hands and feet.
Ovarian failure, coarction of the aorta, horseshoe kidney, recurrent acute otitis media, sensorineural hearing loss, HTN due to renal anomalies, autoimmune disease (thyroid, Celiac), dysgenesis of lymphatic system (causing edema), nail dysplasia.
91
When to suspect hypothyroidism in children:
```
GROWTH FAILURE!!!!!! (most important)
Large tongue
Umbilical hernia
APATHY, Sluggishness, fatiguability
Hypercholesterolemia
Obesity
```
92
Diagnostic evaluation of precocious puberty
Check LH, FSH, androgens, progesterone, estradiol.
Bone age, Lupron (GnRH) stimulation test, pelvic ultrasound.
93
McCune-Albright Syndrome (MAS)
peripheral precocious puberty
café-au-lait skin pigmentation
fibrous dysplasia of bone (polyostotic fibrous dysplasia)
More common in girls than in boys. Affected girls overproduce estrogens, boys-androgens.
94
Endocrine DiffDx for Obesity
Cushing's Disease
Hypothyroidism
Growth hormone deficiency
Endocrine etiologies
95
Lesch-Nyhan Syndrome
X-linked recessive disorder (so only males can get it) resulting from a deficiency in HPRT enzyme (hypoxanthine-guanine phosphoribosyl transferase) which is involved with purine metabolism. This leads to increased levels of uric acid. Presents around 6 months with hypotonia and persistent vomiting. Develop mental retardation, spasticity, dystonia, gouty arthritis and compulsive self injury, especially biting of the upper extremities.
96
Osteogenesis Imperfecta
Defect in type 1 collagen. Type II OI is the most severe form and characteristic findings include blue sclerae, multiple fractures causing limb deformity, and IUGR.
97
Is premature pubarche benign?
No, it is associated with a CNS disorder in 50% of cases and requires a thorough evaluation.
98
What is the most common sequelae associated with Kawasaki disease?
Coronary Artery Aneurism. Prevent this by administering aspirin and IVIG within 10 days of the start of symptoms. Cardiac follow up 2 weeks after disease.
99
Burkitt Lymphoma
Cancer of mature B cells, associated with EBV.
100
Acute Myelocytic Leukemia (AML)
Predominant cancer cells are of myeloid origin. Auer rods are specific for this.
101
Parinaud's syndrome
Presents with paralysis of vertical gaze that may be associated with pupillary disturbances and eyelid retraction (Collier’s Sign). The lesion is most commonly germinomas and pinealomas at the rostral midbrain near the superior colliculus and CNIII. The endocrine syndrome results from interruption of hypothalamic inhibiting pathways and sometimes hCG analogue secretion → Leydig cell stimulation → precocious puberty.
102
Medulloblastoma arises from the ___ 90% of the time.
Cerebellar vermis
103
Fancy way of saying "clubfoot"
talipes equinovarus, equinus and varus of the calcaneum and talus
104
Nikolsky's sign
Slight rubbing of the skin results in exfoliation of the outermost layer, forming a blister within minutes.
Sign is almost always present in toxic epidermal necrolysis (TEN), and is associated with pemphigus vulgaris and Scalded Skin Syndrome (caused by the exfoliative toxin of S. aureus).
105
Pharyngitis etiologies
All viruses associated with URIs
Coxackievirus, EBV, CMV
Bacterial causes: S. pyogenes/GABHS/Strep throat, diptheria
106
GABHS pharyngitis
Seen in school age children 5-15 yo in the winter and spring.
There is a LACK of other URI symptoms, exudates on the tonsils, petechiae on soft palate, strawberry tongue, enlarged cervical lymph nodes.
107
Impetigo
A superficial skin infection affecting the upper dermis, usually caused by S. aureus, but can also be caused by S. pyo. Features: honey-colored crust around the nares, fever absent, very contagious. Treat with topical mupirocin or oral -cillins, third gen. cephalosporins, or clindamycin.
Complications include: bacteremia, post-strep glomerulonephritis (treatment does not prevent this), and SSSS.
108
Erysipelas
Skin infection usually caused by GABHS (80%)
involves the dermal lymphatics
tender, shiny, erythematous skin with a distinct border
Commonly face and scalp
Treat with systemic abx against GABHS.
Complications include bacteremia, post-strep glomerularnephritis, and necrotizing fasciitis.
109
Cellulitis
A skin infection that occurs within the dermis, usually caused by S. aureus or GABHS. Infection is usually caused by a break in the skin barrier. Features: erythema, warmth and tenderness with an INDISTINCT border. Treatment with abx directed at the typical causative agents, with third gen cephalosporins or penicillins.
110
Buccal Cellulitis
Unilateral bluish discoloration on the cheek of a young unimmunized child caused by HIB. High rate of concomitant bacteremia and meningitis.
111
Perianal cellulitis & constipation caused by...
GABHS
112
Staphylococcal Scalded Skin Syndrome (SSSS)
Features: fever, tender skin, bullae, sheets of skin slough off days after beginning of illness. Caused by S. aureus species that produces an exfoliative toxin. Nikolsky sign present (extension of bullae when pressure is applied to the skin).
Treat with IV abx against S. aureus.
113
How do you remember how to diagnose Kawasaki Disease?
"Crash & burn"
1. Conjunctivitis (bilateral, non purulent)
2. Rash ( truncal, non-specific)
3. Adenopathy (at least 1 cervical node > 1 cm)
4. Strawberry tongue (or any change in oropharyngeal mucosa)
5. Hand, feet swelling/desquamation.
Fever (burn) for > 5 days and four or five of the parameters above.
114
Rickettsial Infections (3)
Lyme Disease (Borrelia burgdorferi)
Rocky Mountain Spotted Fever (Rickettsia rickettsii, gram negative)
Ehrlichiosis (Ehrlichia chaffeensis)
115
Rocky Mountain Spotted Fever (RMSF)
Caused by Rickettsia rickettsii, a gram negative intracellular coccobacillus that is transmitted by tick bites, mostly in the SE United States in the spring and summer.
Features: fever, abdominal pain, petechial rash (begins on lower extremities and moves to hands and then trunk and head), palmar rash, myalgias, HSM & jaundice, CNS sx like headache!!!!, hypotension.
Labs: Thrombocytopenia, hyponatrimia, possible aseptic meningitis.
Treat with oral or IV doxycycline (or amoxicillin if under 8 yo) and supportive care.
116
Scrofula/Mycobacterial cervical lymphadenitis
A lymphadenitis of the cervical lymph nodes associated with tuberculosis as well as non tuberculous (atypical) mycobacteria. In immunocompetent children, scrofula is often caused by atypical mycobacteria (Mycobacterium scrofulaceum) and other nontuberculous mycobacteria (NTM). Unlike the adult cases, only 8% of cases in children are tuberculous.
Features: Chronic, painless neck mass that grows. No local color or warmth. Overlying skin acquires a violaceous (bluish-purple) color. As lesion progresses, skin becomes adhered to the mass and may rupture, forming a sinus and an open wound.
Treatment: Surgical excision without antimicrobial therapy. If refractory, treat with a macrolide (clarithromycin or azithromycin) in combination with another agent.
117
Wiskott-Aldrich Syndrome (WAS)
a rare X-linked recessive disease characterized by eczema, thrombocytopenia, immune deficiency, and bloody diarrhea (secondary to the thrombocytopenia). It is also sometimes called the eczema-thrombocytopenia-immunodeficiency syndrome. Initial manifestations at birth consist of petechiae, bruises, bleeding from circumcision or bloody stools.
Not enough platelet PRODUCTION, and the platelets are small.
Combined B and T cell disorder, recurrent infections with encapsulated organisms.
118
Chediak-Higashi Syndrome
A rare autosomal recessive disorder that arises from a mutation of a lysosomal trafficking regulator protein, which leads to a decrease in phagocytosis. The decrease in phagocytosis results in recurrent pyogenic infections (usually S. aureus), partial albinism, peripheral neuropathy, and HSM. Findings of neutropenia and giant lysosomes in neutrophils will confirm the diagnosis. Treatment is with daily trimethoprim/sulfamethoxazole and daily ascorbic acid.
119
Leukocyte adhesion defect (LAD) syndromes
Syndromes that result from failure of innate host defenses against bacteria, fungi and other microorganisms due to defective tethering, adhesion, and targeting of myeloid leukocytes to sites of microbial invasion. The hallmark of the disease is neutrophilia WITHOUT polymorphs in the infected tissue or pus. Pts will typically have a history of delayed separation of the umbilical cord, recurrent bacterial infections, necrotic skin lesions, severe gingivitis, periodontitis, and alveolar bone loss leading to early loss of teeth.
120
Hyper-IgE Syndrome (Job’s)
Syndrome characterized by chronic pruritic dermatitis, recurrent staph infections of the skin and URT, markedly elevated serum IgE levels, eosinophilia and coarse facial features. Recurrent staph infections involving the skin, lungs, and joints, with other features such as distinctive facial appearance, dental abnormalities, and bone fractures are essential for the diagnosis.
121
What is the most common predisposing factor for acute bacterial sinusitis?
A viral upper respiratory infection
122
What is topical erythromycin opthalmic ointment good for prophylactically treating? What does it not work against?
Topical erythromycin (and silver nitrate solution) is only effective for gonococcal prophylaxis (GC shows up at 2-4 days of life). If needed, treat with IV cefotaxime.
Only prenatal maternal testing and treatment are effective for the prevention of neonatal chlamydial infections (4-10 days of life). Discharge may be blood stained. Treat affected infant with oral erythromycin.
123
Osteonecrosis (Avascular necrosis)
A common complication of sickle cell anemia secondary to red cell sickling, microinfarctions, and bone hyperplasia. The most common sites are the humeral and femoral heads. Treatment is pain management and limitation of weight bearing with surgical intervention if conservative management is unsuccessful.
124
Charcot arthritis
Neuropathic arthropathy. Progressive degeneration of a weight bearing joint, a process marked by bony destruction, bone resorption, and eventual deformity, resulting from deterioration of proprioception, pain sensation, and temperature sensation. Onset is usually insidious/gradual.
125
Neuroblastoma (NBL)
The most common pediatric cancer in the first year of life. The median age at diagnosis is 2 years. The tumor arises from neural crest cells from the sympathetic chain or the adrenal medulla. The tumor is usually firm and nodular, and it frequently crosses the midline with systemic symptoms. The levels of serum catecholamines and their metabolites (homovanillic acid and vanillylmandelic acid) are usually elevated. See calcifications and hemorrhages.
126
homovanillic acid and vanillylmandelic acid
Catecholamine metabolytes
127
Metanephros gives rise to...
Renal parenchyma (Wilms tumor can arise from this)
128
Mesonephros gives rise to...
Seminal vesicles, epididymis, ejaculatory ducts, and ductus deferens
129
Paramesonephron gives rise to...
Fallopian tubes, uterus, and part of the vagina.
130
Pyloric Stenosis
Usually presents between 3 and 12 weeks of age with nonbilious, nonbloody, projectile vomiting.
131
Treatment for absence seizures
Ethosuximide or valproic acid (valproate)
132
Treatment for partial seizures
Phenytoin or Gabapentin
133
Is Staph aureus resistant to penicillin?
Yes, >99% of S. aureus is resistant to penicillin. Use clindamycin or vancomycin.
134
Treatment for facial impetigo
Topical mupirocin (or oral erythromycin if it's bad)
135
In what ways can diabetes evoke an immunodeficient state?
Diabetes Mellitus can evoke an immunodeficient state when you have lots of hyperglycemia. The hyperglycemia promotes neutrophil dysfunction, and circulatory insufficiency contributes to ineffective neutrophil chemotaxis during infection.
136
LAD/leukocyte adhesion deficiency
Disorder of leukocyte adherence
Recurrent sinopulmonary, oropharyngeal, and cutaneous infections
Delayed wound healing (delated umbilical detachment, gingivitis)
Neutrophilia, high WBC >50,000
ABSENCE of neutrophils from infection sites!
Life-threatening infection is possible with Staph spp., Enterobacteriaceae, and Candida spp.
137
Severe Combined Immunodeficiency (SCID)
An autosomal recessive or X linked disorder of both humoral and cellular immunity. Serum immunoglobulins and T cells are often markedly diminished or absent. Thymic dysgenesis is also seen. Recurrent cutaneous, GI, or pulmonary infections occur with opportunistic organisms like CMV and PCP. Death usually occurs in the first 2 years of life unless a curative bone marrow transplant is performed.
138
DiGeorge Syndrome
Syndrome caused by a 22q11 microdeletion, leading to immunodeficiency.
```
CATCH-22 mnemonic:
Cardiac Abnormality (especially tetralogy of Fallot)
Abnormal facies
Thymic aplasia
Cleft palate
***Hypocalcemia***/Hypoparathyroidism
```
```
Also:
decreased T cell production (IMMUNODEFICIENCY)
recurrent infection
VSD and Tetralogy of Fallot
Thymic or parathyroid dysgenesis
hypocalcemia
seizures
Developmental and speech delay
```
139
What is the most common inherited form of mental retardation?
Fragile X Syndrome
140
Klinefelter Syndrome
XXY male. Features mild mental delay, gynecomastia, delayed puberty, sterility, increased rates of breast cancer, small testes and phallus.
141
Goat-milk diet: nutritional concerns
Can have folate or B12 deficiency leading to megaloblastic anemia. It must also be boiled before ingestion, as goats are susceptible to brucellosis.
142
Normal total bili (excluding newborns)
Less than 1.2 mg/dl
143
Choledochal cyst
A congenital abnormality of the biliary ducts characterized by the dilation of intra and/or extrahepatic biliary ducts or both. It presents in children with abdominal pain, jaundice and attacks of recurrent pancreatitis (may increase amylase and lipase levels). They will have epigastric RUQ pain. These cysts can degenerate into cholangiocarcinomas.
144
Cholesteatoma
a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. It can be congenital or acquired. You can note granulation tissue and skin debris within retraction pockets of the tympanic membrane. Should be suspected in any patient with continued ear drainage for several weeks despite appropriate antibiotic therapy. Complications include hearing loss, cranial nerve palsies, vertigo, and severe infections.
145
Normal ESR Ranges
Newborn: 0 to 2 mm/hr
| Neonatal to puberty: 3 to 13 mm/hr
146
Normal CRP Value
<0.8 mg/dL)
147
Diagnostic Criteria for TSS
Causative Agents: S. aureus, less common is GABHS.
1. Fever > 38.5 (101)
2. Hypotension
3. Diffuse macular erythroderma, like a sunburn
4. Desquamation 10-14 days after illness onset.
5. Multisystem involvement (>3 of the following: GI, myalgias, hyperemia of mucus membranes, sterile pyuria, thrombocytopenia, CNS altered consciousness)
6. Negative cultures of blood, CSF, pharynx, OTHER than blood culture for S. aureus.
148
Viral diarrhea
```
Rotavirus (4-7 days, self limiting, supportive care)
Norwalk virus (2-3 days, daycares/cruises/etc., supportive care)
```
149
Diarrhea causes what kind of metabolic derangement?
non-anion gap hyperchloremic metabolic acidosis, as a result of bicarbonate loss in the stool
150
Which organism causes hemolytic uremic syndrome?h
Enterohemorrhagic E. coli (EHEC), via endotoxin release. If HUS is present, DON'T use antibiotics.
151
Bloody diarrhea differential
Shigella, Salmonella, Campylobacter (most common)
152
At what age is a negative HIV-specific DNA PCR definitive for not having congenitally-acquired HIV?
4 months
153
Early symptoms of HIV infection
```
FTT
thrombocytopenia
recurrent infections (otitis media, pneumonia, and sinusitis)
lymphadenopathy
parotitis
recurrent, difficult to treat Thrush
loss of developmental milestones
severe varicella infection or zoster
```
154
Which vaccines CAN'T an HIV positive child get and why?
They cannot get the varicella vaccine or the MMR vaccine (well, some can). This is because the vaccines are live.
155
Common infections of HIV+ persons
PCP
M. avium complex (MAC)
Fungal: Candida, cryptococcus, histoplasmosis, coccidioidomycosis, aspergillosis.
Viral: CMV, HSV, VZV
Parasites: Toxoplasmosis, cryptosporidium
Lymphoma (especially B cell, caused by EBV co-infection)
156
Major etiologic agent of mononucleosis
EBV, transmitted by saliva and infects the B lymphocyte.
157
EBV infectious mononucleosis symptoms
```
Fever, may last up to 2 weeks
Malaise and fatigue
Pharyngitis (typically exudative, resembles GABHS)
Posterior cervical lymphadenopathy
HSM (80%)
macular or scarlatiniform rash
```
Diagnose with monospot test (same thing as heterophile antibody test)
Sx resolve in weeks to months
158
How do you test for EBV infectious mononucleosis?
Monospot in children >4 yo. Monospot measures the presence of heterophile antibody, with a sensitivity of 85%.
For children <4 yo., use EBV antibody titers.
159
EBV Infectious Mononucleosis treatment
Supportive
160
A patient you think has GABHS pharyngitis is administered amoxicillin and breaks out in a diffuse pruritic maculopapular rash. What has happened?
This patient has an EBV infection and was misdiagnosed. We don't know why, but patients who have EBV infectious mononucleosis who are mistakenly administered amoxicillin break out in this rash. This is not an allergic reaction.
161
Another name for measles:
Rubeola
162
Classic prodrome of measles virus
The three C's: cough, conjunctivitis, coryza (stuffed nose).
163
Describe the measles exanthem.
- THREE C'S Prodrome: cough, conjunctivitis, coryza (stuffed nose).
- Oral exanthem prodrome
- Koplik's Spots (blue-gray papules on a red base on inner cheek), pathognomonic
- brick-red skin exanthem maculopapular eruption (face-->downwards)
- Lasts for 4-7 days.
164
Measles complications
Bacterial pneumonia (most common)
Otitis media
Laryngotracheitis
Encephalomyelitis (infl. of both brain and cord)
165
Rubella clinical features
Incubation 14-21 days.
Prodrome of mild URI symptoms and low fever.
Painful lymphadenopathy (at suboccipital, posterior, auricular, and cervical nodes).
Exanthem: NONPRURITIC maculopapular and confluent. Begins on FACE and spreads to trunk and extremities. Lasts 3-4 days.
166
Congenital Rubella Syndrome (CRS)
Infection of mother in the 1st trimester will cause fetal anomalies in 30-50% of fetuses. This includes HSM, jaundice, purpura (blueberry muffin baby). Congenital cataracts, PDA, and sensoruneural hearing loss.
167
Invasive Aspergillosis treatment
High dose amphotericin B antifungal therapy, surgical resection of the aspergilloma. Occurs in immunocompromised. Poor prognosis.
168
Coccidioidomycosis etiology and treatment
A fungus found in the soil in the SW United States and Mexico. Treat disseminated disease or disease in the immunocompromised with systemic antifungal therapy.
169
Cryptococcus neoformans etiology and features
A yeast found in the soil, acquired when the yeast is inhaled into lungs. Most infections are asymptomatic.
Disseminates in the immunocompromised, rare in children.
Treat with systemic antifungal therapy.
170
How does Factor VIII deficiency affect PT and PTT?
Factor VIII deficiency prolongs the PTT but not the PT.
171
Most common nutritional deficiency in children
Iron
172
Osteomyelitis in the setting of sickle cell disease is most likely caused by...(2)
S. aureus and Salmonella
173
What are the most common causes of septic arthritis and osteomyelitis in neonates? (2)
GBS and E coli. GBS is the most common cause of osteomyelitis in children <1 yo.
174
Pseudomonas osteomyelitis is seen in who?
diabetic patients or after puncture wounds of the foot
175
Asplenia in sickle cell patients makes them more susceptible to what?
Encapsulated organisms. S. pneumoniae is the most common cause of bacteremia in these children, but it typically causes meningitis and pneumonia, not really osteomyelitis.
176
Most common cause of meningitis for children and adults in the US
Neisseria meningitides, with a mortality rate of 15%.
177
Another name for meningococcus
Neisseria meningitides
178
Causative organism of a meningitis + petechial/purpuric rash =
Neisseria meningitides (meningococcus). Petechial rash will be on the axilla, wrists, flanks, and ankles.
179
Brudzinski sign
in meningitis, flexion of the neck usually causes flexion of the hip and knee.
180
CSF in bacterial meningitis will show:
Increased WBC count with a neutrophil predominance, increased protein, and decreased glucose.
181
Most common causes of viral meningitis:
Non-polio enteroviruses, such as echovirus and coxackievirus.
182
Congenital rubella syndrome
sensorineural deafness, cataracts, HSM, purpura.
183
Phenytoin (antiepileptic) exposure en utero results in...
hypoplastic fingers and nails, and cleft lip/palate. This is called fetal hydantoin syndrome.
184
2 most common causes of acute bacterial rhinosinusitis
Strep pneumo and Hib
185
Leading Cause of congenital deaf-blindness?
CHARGE Syndrome
186
What is CHARGE syndrome?
C - Coloboma of the eye, central nervous system anomalies
H - Heart defects
A - Atresia of the choanae
R - Retardation of growth and/or development
G - Genital and/or urinary defects (Hypogonadism)
E - Ear anomalies and/or deafness
187
Single umbilical artery is associated with what?
Heart defects
Renal defects
Trisomy 18
188
When do fetuses start producing surfactant?
32 weeks gestation. The occurrence of RDS greatly declines at this time.
189
Hepatitis B is most common in what racial group?
Asians. Over 50% of US cases are Asians.
190
What keeps the ductus arteriosus open?
PGE1 (prostaglandin E1)
191
What can close a PDA?
NSAIDs like ibuprofen (they block PGE1, which keeps the DA open), also INDOMETHACIN (PGE inhibitor)
192
What fetal anomaly is associated with mothers who have SLE?
Congenital third degree AV block.
193
Maternal tobacco use during pregnancy increases risk for...
Low birth weight (<2500)
194
Maternal diabetes increases risk for...
Large for gestational age (LGA)/macrosomia
195
Components of the APGAR score
```
Appearance: blue to pink
Pulse: under 100 to over 100
Grimace
Activity (tone)
Respiration: absent to good cry
```
196
SGA newborns are at risk for...(3)
Hypoglycemia, hypothermia, and polycythemia.
197
Newborn born to an HIV+ mother. What 3 precautions do you take?
1. Bath baby before any injections.
2. No breastfeeding.
3. Zidovudine should be initiated by 12 hours of life
198
Can jaundice in the first 24 hours be benign?
No. It warrants investigation.
199
How many kcal of formula do healthy babies need per day?
About 120 kcal/kg/day for the first year of life, and 100 after that.
200
Can mothers with Hepatitis B breastfeed safely?
Yes.
201
What hormones are involved with milk production and let down?
Prolactin is involved in milk production, while oxytocin acts to cause milk ejection which is often called "let-down."
202
What is galactosemia?
Can't metabolize galactose, a metabolic product of lactose breakdown. Resulting in hepatomegaly (an enlarged liver), cirrhosis, renal failure, cataracts, brain damage, and ovarian failure. Without treatment, mortality in infants with galactosemia is about 75%. Autosomal recessive. The only treatment for classic galactosemia is eliminating lactose and galactose from the diet.
203
Breast Milk Jaundice
Diagnosed in a healthy infant with good weight gain who at >7 days is jaundiced, has a normal direct bilirubin level and an elevated total bilirubin level. The etiology appears to be an unknown substance in maternal milk that causes increased reabsorption of bilirubin from the stool and decreased excretion of bilirubin.
204
How long should it take for a baby's weight to double? Triple? Quadruple?
Birth weight should double by 4-5 months.
Triple by 1 year.
Quadruple by 2 years.
205
Which vaccines are given at 1 year?
MMR and HepA
206
When do babies first develop object permanence?
9-10 months
207
What is the most common cause of a left-sided abdominal mass?
Constipation
208
What test is highly specific for neuroblastoma?
Urine or serum Vanillylmandelic Acid and Homovanillic Acid (VMA/HVA), measures metabolites of catecholamines.
209
DKA signs and symptoms (7)
```
Fruity breath
Vomiting
Weight loss
Dehydration
Shortness of breath
Abdominal pain
Change in the level of consciousness
```
210
Signs of cerebral edema and increased intracranial pressure
Inappropriate slowing of heart rate, rising blood pressure, irregular breathing (Cushing reflex)
211
Having Type 1 DM puts you at higher risk for...
other autoimmune diseases like Celiac or Thyroid disease
212
NF1
Autosomal dominant genetic disorder with highly variable manifestations, including skin hyperpigmentation lesions (cafe au lait macules), multiple benign cutaneous nerve sheath tumors (neurofibromas), axillary and inguinal freckling, macrocephaly, iris hamartoma (Lisch nodules). Common symptoms of NF-1 include scoliosis (curvature of the spine), learning disabilities, vision disorders, and epilepsy.
213
Most common drugs causing allergic reactions
penicillins
sulfonamides (including most loop and thiazide-type diuretics)
phenytoin (antiseizure)
214
Hand, Foot, Mouth Disease (etiology, signs and symptoms, treatment)
Etiology: Coxsackievirus A. Highly contagious viral infection characterized by fever, painful oral lesions, and rash on hands and feet (and butt). Occurs in children under 10. Transmitted oral-oral or fecal-oral. 12-36 hour prodrome may develop with fever after incubation period; painful oral lesions may appear after 1-2 days, followed by painful skin rash. Supportive treatment, usually lasts 13 days.
215
Roseola
AKA Sixth Disease, HHV6 and HHV7.Ages 6 months-2 years. Sudden high fevers and just as the child appears to be recovering, a red rash appears. This usually begins on the trunk, spreading to the legs and neck. The rash is not itchy and may last 1 to 2 days. No vaccine. Supportive treatment.
216
Causes of palpable purpura (7)
```
Rocky mountain spotted fever
Acute meningococcemia
Disseminated gonococcal infection
Ecthyma gangrenosum
Henoch Schonlein purpura
Polyarteritis nodosa
Leukocytoclastic vasculitis
```
217
What is silver stain used for?
Fungi (like aspergillus, pneumocystis, and candida)
218
Fever definition
Fever >38C (100.4F)
High fever is >39C (102.2F)
219
When is the highest incidence for bacterial meningitis?
In the first month of life.
220
What do you treat disseminated herpes with?
Acyclovir (it's nephrotoxic though)
221
How do you treat acute (bacterial) sinusitis?
With amoxicillin, amox-clavulanate or second gen-cephalosporin for 10-14 days.
222
How do you treat chronic sinusitis?
With broad-spectrum abx, and IV abx may be beneficial.
223
Strep throat (Bacterial pharyngitis).
Cause?
How is it different from other URIs?
Treatment?
Caused by GABHS.
LACKS OTHER URI SX like rhinorrhea and cough, has exudates on the tonsils, petechiae on the soft palate, strawberry tongue, and enlarged tender anterior cervical lymph nodes.
Treat with oral penicillin VK and IM benzathine penicillin. If allergic, give oral erythromycin or macrolides.
224
How do you treat GERD?
Ranitidine (trade name Zantac) is a histamine H2-receptor antagonist that inhibits stomach acid production to treat GERD.
225
What is Periventricular leukomalacia and who does it mostly affect?
Periventricular leukomalacia (PVL) is a form of white-matter brain injury, characterized by the necrosis (more often coagulation) of white matter near the lateral ventricles, affecting premature infants. Exhibit motor control problems, developmental delays, cerebral palsy or epilepsy later in life.
226
Treatment for Lyme Disease in children under 8
Amoxicillin (NOT DOXY!)
227
What does a "left shift" mean on WBC counts?
The presence of increased numbers of bands (immature WBCs) usually indicates a response to inflammatory cytokines. There are usually 2-6% bands. This may be due to an active infection. "Segs" are mature cells.
228
Sickle cell patients lose their spleen due to microinfarctions by what age?
2-3 years. This makes them susceptible to encapsulated organisms like:
```
Haemophilus influenzae type b (Hib)
Streptococcus pneumoniae (pneumococcus)
Neisseria meningitidis (meningococcus)
Group B streptococcus (GBS)
Klebsiella pneumoniae
Salmonella typhi
```
229
Klippel-Feil Syndrome
A failure of normal vertebral segmentation that results in relative fusion of the involved vertebrae, often occurring the the cervical spine. Can also be associated with congenital torticollis, GU anomalies, congenital heart disease, hearing loss, and congenital abnormality of the scapula (Sprengel’s deformity).
230
Spondylolysis
A stress fracture in the pars interarticularis, the bone that connects the superior and inferior articular facets of a vertebral body, occurring with repetitive hyperextension of the spine (gymnastics, tennis, diving). Treat with rest, analgesics, and possibly casting.
231
Spondylolisthesis
Occurs when the body of the vertebra involved in spondylolysis slips anteriorly (subluxation of the vertebra).
232
When does progression of scoliosis occur?
Only during growth or if the spinal curvature is greater than 50 degrees.
233
Transient synovitis
A common self-limited postinfectious response of the hip joint. URIs or diarrhea often precede transient synovitis. This is a diagnosis of exclusion and you need to first rule out septic arthritis.
234
Septic Arthritis
a bacterial infection of the joint, usually occurring on ages 1-4, mostly in the hip. Common organisms are S. aureus, Strep pyo (GABHS), and Neisseria gonorrhoeae in adolescents. Treat with joint aspiration and drainage, empiric IV antibiotics to cover gram-positive organisms.
235
Legg-Calve-Perthes Disease
Idiopathic avascular necrosis of the femoral head. Occurs in ages 4-9 yrs in caucasians and Asian boys (M:F, 4:1). Diagnosis is by seeing the “crescent sign” on radiograph, indicating a crescentic subchondral fracture in the femoral head. Treatment is containment, PT, and restriction of vigorous activity.
236
Fluoroquinolones
CONTRAINDICATED IN CHILDREN DUE TO CARTILAGE DESTRUCTION
broad-spectrum antibiotics (effective for both gram-negative and gram-positive bacteria). Ex. Ciprofloaxacin
237
Osteomyelitis
An infection of the bone. Peak Ages are less than 1 year. Most commonly caused by S. aureus and GABHS. Salmonella should be considered in a SS patient. On labs. you would see high WBC, ESR, and CRP. Treat with antibiotics (fluoroquinolones and beta-lactam antibiotics) for 6 weeks. May see pathologic fractures.
238
Genu varum
Bowed legs
239
Genu valgum
knock-knees
240
Osgood–Schlatter disease
"Traction apophysitis"
An irritation of the patellar ligament at the tibial tuberosity characterized by painful lumps just below the knee and is most often seen in young adolescents, aged 10-17. Risk factors may include overzealous conditioning (running and jumping), but adolescent bone growth is at the root of it. Pain occurs with extension of the knee against resistance. Manage with rest, analgesics, and stretching.
241
Salter-Harris Fracture Types
Salter-Harris Fractures involve the growth plate (physis). Remember the acronym: SALTR
Grades
1-S-same, fracture within the physis
2-A-above, fracture in the physis and metaphysis
3-L-low, fracture in the physis and epiphysis
4-T-through, fracture in the physis, metaphysis, and epiphysis
5-R-cRush, crushing of the physis
242
Toddler's Fracture
Spiral fracture of the tibia, which may occur after very mild or no identified trauma. Usually occurs between 9 months and 3 years of age, when a child trips and falls.
243
Diagnostic value of CRP levels
Normal concentration in healthy human serum is usually 0-10 mg/L (most under 3).
Higher levels are found in mild inflammation and viral infections (10–40 mg/L), active inflammation, bacterial infection (40–200 mg/L), severe bacterial infections and burns (>200 mg/L).
CRP is a more sensitive and accurate reflection of the acute phase response than the ESR.
244
Foot drop differential for kids
muscular dystrophy (DMD, BMD, MMD)
Charcot Marie Tooth disease
cerebral palsy
Friedreich's ataxia
245
Charcot Marie Tooth disease
Hereditary neurologic disorder (17p11.2 duplication). Progressive loss of muscle tissue and touch sensation across various parts of the body, decreased muscle tone, distal muscle wasting.
246
Marfan Syndrome
Autisomal dominant disorder of the connective tissue, defect in fibrillin1 gene (FBN1) on chromosome 15. People with Marfan tend to be unusually tall, with long limbs and long, thin fingers. Features: retinal detachment, aortic root dilation, crowded teeth, lens dislocation.
247
Williams Syndrome
```
7q11 deletion
"elfin" facial appearance
low nasal bridge
cheerful demeanor and ease with stranger
developmental delay coupled with strong language skills
supravalvular aortic stenosis
hypercalcaemia
```
248
Prader-Willi Syndrome
```
15q11.2 deletion of the father
Decreased muscle tone
small genitals
obesity
mental retardation/dev. delay
almond shaped eyes
downturned mouth
temper tantrums
```
249
Beckwith-Wiedemann syndrome
Large tongue, tissue and organ overgrowth
Mild mentral retardation
Hemihyperplasia
Wilm's Tumors
250
Positive urine nitrite test indicates...
Gram negative UTI, like for E. coli
251
First step in a child with language delay
Audiology screen
252
Proximal RTA (Type 2)
Failed HCO3− reabsorption from the urine by the proximal tubular cells.
Urine CAN acidify to a pH of less than 5.3.
Can present in Fanconi Syndrome (proximal tubule dysfunction).
Hypokalemia
Treat with oral bicarbonate supplementation.
253
Distal RTA (Type 1)
Failure of H+ secretion by the α intercalated cells.
Features:
inability to acidify the urine to a pH of less than 5.3
normal anion gap metabolic acidosis/acidemia
Hypokalemia
***Urinary stone formation***
Bone demineralization (causing rickets in children and osteomalacia in adults)
Treat with sodium citrate.
254
What does an elevation in alkaline phosphatase (AP), bilirubin and gamma-glutamyl-transpeptidase (GGT) suggest?
These elevations often suggest cholestatic injury.
AP can also be high with bone regeneration, and GGT will be normal in this scenario.
255
Monospot test tests for...
EBV
256
Azithromycin
Use for chlamydia, gonorrhea, H. influenzae, Streptococcus pneumoniae, Streptococcus pyogenes
257
Precocious puberty ages
7 for girls
| 9 for boys
258
Normal alkaline phosphatase
The normal range is 44 to 147 IU/L.po
High levels of ALP are normally seen in children undergoing growth spurts.
```
Higher-than-normal ALP levels
•Biliary obstruction
•Bone conditions
•Osteoblastic bone tumors, osteomalacia, a fracture that is healing
•Liver disease or hepatitis
•Eating a fatty meal if you have blood type O or B
•Hyperparathyroidism
•Leukemia
•Lymphoma
•Paget's disease
•Rickets
•Sarcoidosis
```
259
Are chlamydia and gonorrhea gram-positive or gram-negative?
Gram negative.
260
How do you treat chlamydia and gonorrhea?
ORAL azithromysin (Chl) and IV ceftriaxone (Gonorr). Can also use topical erythromycin for gonorrheal conjuntivitis.
261
What kind of drug is Enalapril?
ACE inhibitor
262
Alport syndrome
```
A genetic defect in type IV collagen in the GBM.
X linked dominant
Progressive nephritis
Hearing loss
Ocular abnormalities
```
263
Chronic Granulomatous Disease
Disease of immunodeficieny. Defect of phagocytic cells due to dysfunction of the NADPH oxidase enzyme complex.
Recurrent and uncontrolled infections:
pneumonia
abscesses of the skin, tissues, and organs
suppurative arthritis
osteomyelitis (multiple sites)
bacteremia/fungemia
superficial skin infections such as cellulitis or impetigo
The diagnosis is made by nitroblue tetrazolium (NBT) slide test, flow cytometry, or cytochrome C reduction. Treatment is with daily trimethoprim/sulfamethoxazole and IFNy 3 times a week.
264
Empiric skin infection antibiotic
Clindamycin
265
What is the #1 cause of blindness worldwide?
Cataracts. corrective surgery is imperative in children so their visual cortex can form (prevent ambylopia). Perform before 2-3 months!
266
Retinoblastoma (genetic etiology, age group)
non-functional growth suppressor gene on chromosome 13.
13-18 months (preschool age).
See calcifications within the tumor.
Increases risk of osteoscarcoma.
267
What is a 10% potassium hydroxide (KOH) test used for?
Idenitification of fungal hyphae on scales or scrapings.
Orgs include Tinea, Candida (yeast).
268
Down Syndrome, DiGeorge & CHARGE syndrome cardiac finding
Tetralogy of Fallot (squat to relieve symptoms)
269
Seborrheic Dermatitis (ages, etiology, features)
Also called cradle cap.
| Ages: infants and adolescents.
270
Gilbert Syndrome
Genetic liver disorder. Most common hereditary cause of increased bilirubin.
Features jaundice due to elevated levels of unconjugated bilirubin (hyperbilirubinemia)
Caused by reduced activity of glucuronyltransferase, doesn't need treatment.
271
A baby is delivered by an untrained birth attendant and mom had no prenatal care. At 14 days he is seizing. Why?
He has a tetanus infection.
Neonatal tetanus is a form of generalized tetanus that occurs in newborns. Infants who have not acquired passive immunity because the mother has never been immunized are at risk. It usually occurs through infection of the unhealed umbilical stump, particularly when the stump is cut with a non-sterile instrument.
Significant problem in developing countries with unimmunized mothers (no passive immunity).
272
Precocious puberty ages
7 for girls
| 9 for boys
273
ALL diagnostic finding
The presence of >25% lymphoblasts in bone marrow.
Lymphoblasts often contain cytoplasmic aggregates of periodic acid Schiff (PAS) positive material. Immunostaining for TdT is positive in more than 95% of patients (TdT is only expressed in pre B and preT lymphocytes).
```
Also:
Thrombocytopenia (see petichiae)
Pallor
Weightloss
Rubbery lymphadenopathy
```
274
Nevus simplex
```
Salmon patch birthmark
Common
Benign
blanchable
caused by dilation of certain blood vessels
Goes away with age
```
275
Nevus flammeus
Port-wine Stain
Rare
benign vascular tumor, not true neoplasm, hamartoma
Associated with Sturge-Weber Syndrome (seizures, glaucome, retardation)
276
Sturge-Weber Syndrome
Nevus flammeus along ophthalmic division of the trigeminal nerve, intracranial leptomeningeal angiomatosis ipsilateral to skin lesion, seizure, vascular headache, developmental delay and mental retardation, glaucoma, hemianopsia, hemiparesis.
277
Alport syndrome
A genetic defect in type IV collagen in the GBM.
278
Chronic Granulomatous Disease
Disease of immunodeficieny. Defect of phagocytic cells due to dysfunction of the NADPH oxidase enzyme complex.
Recurrent and uncontrolled infections:
pneumonia
abscesses of the skin, tissues, and organs
suppurative arthritis
osteomyelitis (multiple sites)
bacteremia/fungemia
superficial skin infections such as cellulitis or impetigo
The diagnosis is made by nitroblue tetrazolium (NBT) slide test, flow cytometry, or cytochrome C reduction. Treatment is with daily trimethoprim/sulfamethoxazole and IFNy 3 times a week.
279
What is the most common predisposing factor for acute bacterial sinusitis?
A viral upper respiratory infection.
280
Congenital Adrenal Hyperplasia
What enzyme is deficient?
What builds up?
What are the effects?
21-hydroxylase deficiency, which prevents progesterone and 17-hydroxyprogesterone from being converted into aldosterone and cortisol, respectively. This leads to a mineralocorticoid deficit (hypotension, hyponatremia, hyperkalemia), while the excess 17-hydroxyprogesterone is shunted toward adrenal androgen synthesis, causing virilization in females.
281
Acquired aplastic anemia
Results from an injury to bone marrow by radiation, drugs, insecticides, toxins, or infection.
Sx: pallor, fatigue, weakness, loss of appetite, easy bruising, petechiae, fever.
Bone marrow biopsy is essential to make a diagnosis--shows profound hypocellularity.
282
Diamond-Blackfan Anemia
Congenital pure red cell aplasia.
Presents in the first 3 months of life with pallor and poor feeding.
283
Red flags for increase intracranial pressure (4)
Morning vomiting
Nocturnal headaches
Seizures
Papilledema
284
What are the two common Hemophilias?
A and B.
A: Factor 8 deficiency
B: Factor 9 deficiency
285
What is nortryptiline? What does an OD look like on it? What's the treatment?
A tricyclic antidepressant. If a child ODs on it, you can see seizures, hypotension, and prolonged QRS complexes.
Treatment for this is SODIUM BICARBONATE to correct acidosis and to narrow QRS prolongation.
286
A baby is delivered by an untrained birth attendant and mom had no prenatal care. At 14 days he is seizing. Why?
He has a tetanus infection.
Neonatal tetanus is a form of generalized tetanus that occurs in newborns. Infants who have not acquired passive immunity because the mother has never been immunized are at risk. It usually occurs through infection of the unhealed umbilical stump, particularly when the stump is cut with a non-sterile instrument.
Significant problem in developing countries with unimmunized mothers (no passive immunity).
287
3 markers used to detect trisomies
AFP
unconjugated estriol
b-HCG
288
When is the meningococcal vaccine given?
11-12 years old. (2 yo for asplenic kids)
289
When is the rotavirus vaccine given?
2 months and 6 months. None beyond 8 months.
290
Normal percentage of neutrophils in synovial fluid?
25%
291
Most common organisms causing cervical lymphadenitis (2)
S. aureus
| GABHS
292
What does lymphadenopathy caused by non-TB mycobacterium loparok like?
most commonly myco. avium.
```
<5 yo
firm, nontender
Less than 4 cm
thin skin over lymph node
violacious color
NO FEVER or tenderness
```
293
What is hydroxyurea and what does it do?
It is a chemotherapy agent used in SCD to decrease vasoocculusive pain crises and acute chest syndrome. It increases fetal hemoglobin.
Side effects: bone marrow suppression.
294
SIgns of a pathologic heart murmur (3)
Increases intensity when standing or Valsalva
Any diastolic murmur
Grade 3/6 or higher
295
Can you use cipro in kids?
No. It causes cartilage breakdown and growth retardation.
296
Type 2 Vitamin D dependent Rickets
Disfunctional Vitamin D receptor. See rickets in the setting or normal Vit. D
297
Diamond-Blackfan Anemia
Congenital pure red cell aplasia.
Presents in the first 3 months of life with pallor and poor feeding.
298
Red flags for increase intracranial pressure (4)
Morning vomiting
Nocturnal headaches
Seizures
Papilledema
299
What are the two common Hemophilias?
A and B.
A: Factor 8 deficiency
B: Factor 9 deficiency
300
At what age should babies sit alone?
6 months
301
Normal BUN
6-20
302
3 markers used to detect trisomies
AFP
unconjugated estriol
b-HCG
303
Nesidiobastosis
Islet cell hyperplasia
304
Acro means...?
Distal (acromelia is anomaly of distal bones)
305
Fever and URI with conjunctival injection is caused by what?
Adenovirus, and it's highly contagious! You can detect it in urine.
306
Osteosarcoma
Most common primary bone tumor affecting children and adolescents.
Highest risk is boys 13-16yo.
Occurs at metaphysis of long bones.
No constitutional symptoms.
Startburst pattern on xray.
307
Fanconi Anemia
Congenital cause of aplastic anemia due to chromosomal breaks.
```
Features:
progressive bone marrow failure (thrombocytopenia)
poor growth
hypo/hyperpigmented areas of skin
large freckles
strabismus, low set ears
macrocytic anemia
```
308
What is hydroxyurea and what does it do?
It is a chemotherapy agent used in SCD to decrease vasoocculusive pain crises and acute chest syndrome. It increases fetal homoglobin.
Side effects: bone marrow suppression.
309
SIgns of a pathologic heart murmur (3)
Increases intesity when standing or Valsalva
Any diastolic murmur
Grade 3/6 or higher
310
What does a Holter monitor do?
It is used for outpatient detection of arrhythmias.
311
Type 2 Vitamin D dependent Rickets
Disfunctional Vitamin D receptor. See rickets in the setting or normal Vit. D
312
What vitamin has been shown to reduce morbidity and mortality of children with measles?
Vitamin A, through immune enhancement.
313
Phenylketonuria
An autosomal recessive disorder more freq in the White and Asian populations. Deficieny in phenylalanine hydroxylase, which breaks down phenylalanine into tyrosine, leading to an accumulation of phenylalanine.
This accumulation can result in mental retardation & seizures.
Diagnose with the Guthrie test.
314
Cephalohematoma
A subperiosteal hemorrhage that is localized to the cranial bone that was traumatized during delivery. The swelling does not extend across a suture line. As the blood is reabsorbed from the cephalohematoma it will contribute to hyperbilirubinemia.
315
Caput succedaneum
An edematous swelling over the presenting portion of the scalp of an infant. It overlies the periosteum and the swelling crosses suture lines. The swelling consists of serum and would not cause hyperbilirubinemia. Caused by suction.
316
Mannitol
Diuretic
317
What are the following features of?
Fixed dilated pupil, contralateral hemiparesis, Cushing triad: bradycardia, HTN, irregular breathing
Brain herniation
318
What do these symptoms signify?
headache, pupillary changes, altered mental status, vomiting, loss of vision, gait disturbance, retinal hemorrhage
Increased ICP
319
Can you use activated charcoal for an iron poisoning?
No! Chelate with deferoxamine or do whole bowel irrigation.
320
What is omeprazole?
Proton Pump Inhibitor for GERD, dyspepsia, stomach ulcers
321
Which is more posterior, the trachea or esophagus?
the esophagus
322
What is 5th disease?
parvovirus B19 or Erythema infectiosum. Slapped cheeks and fever, lacy rash, myalgias. Can also cause aplastic crises in SS anemia or HS.
Infection can cause miscarriage or fetal hydrops!!!
323
Roseola/Sixth disease exantham
AFTER high fever for 3-5 days. Can also see febrile seizures.
324
High hemagglutination inhibition antibody titers indicate what?
Measles!
325
Croup is usually caused by what?
Parainfluenza virus.
326
What kinds of infections does H. flu cause in kids?
meningitis (neonates), otitis media, bronchitis, pneumonia, epiglottitis.
327
CD19 cells, aka
B lymphocytes
328
X-linked agammaglobulinemia
B cell deficient immunedeficiency. Present with recurrent LRT and URT bacterial infections 6-18 months old.
Patients receive regular infusions of IVIG to fight bacteria.
329
CD3 cells, aka...
all T cells
330
A child with meningitis becomes septic, develops a purpuric rash, becomes quickly hypertensive, and dies. What happened?
She had Waterhouse-Friderichsen Syndrome, which is sudden vasomotor collapse and purpuric skin rash. 100% fatality.
331
What are Howell Jolly bodies and when do you see them?
They are nuclear remnants for RBCs seen on peripheral blood smears of SS patients. This indicates functional asplenia. Located in the center of RBCs.
332
What are Heinz bodies and when do you see them?
They are aggregates of denatured hg, seen in patients with hemolysis due to G6PD def. and thalassemia. Located on outer edge of RBCs.
333
Infant consumption of honey leads to...
botulism--one cause of floppy baby syndrome (blocks ACh release)
```
Constipation
Symmetric paralysis
Ptosis
Poor feeding
loss of deep tendon reflexes
respiratory difficulties
```
334
Floppy baby syndrome
Werdnig-Hoffman Syndrome, degeneration of the anterior horn cells and CN motor nuclei.
335
Long term sequelae of bacterial meningitis (5)
```
hearing loss
loss of cognitive functions
seizures
mental retardation
spasticity or paresis
```
336
Secondary injury associated with suprachondylar fractures?
Brachial artery injury.
337
Congenital Myotonic Dystrophy features
Autosomal DOMINANT
Inability to relax
Distal muscle weakness
See in second decade
338
Werdnig-Hoffman
```
Spinal muscular atrophy (SMA)
Weak cry, tongue fasciculations, frog leg posture
anterior horn cell degeneration
BEFORE 6 months of age
autosomal recessive
no cure
```
339
Most common form of migraine in children
bifrontal migraine without aura. can use triptans (5HT agonists for cranial vasoconstriction) if acetaminophen and supportive therapies don't work.
340
Acute Cerebellar Ataxia features
most common cause of ataxia in children
trunkal ataxia, slurred speech, nystagmus
no fever
most common cause is varicella (or EBV as well)
affects kids under 10yo
341
Tourette's treatments
Pimozide
Clonidine
Haloperidol
342
Smooth red tongue
B12 deficiency (also ataxia, hyporeflexia, +Babinsky)
343
Sickle cell substitution
AA substitution of glutamic acid to valine (#6 position, B-Hb chain). Not symptomatic until HbF declines at 6 months.
344
Treatment of epilepsy
Generalized: Valproic acid or Phenobarbitol
Absence: ethosuximide
Partial: carbamazepine or phenytoin
345
Factor VIII deficiency name and treatment
Hemophilia A
| Treatment: DDAVP (vasopressin, can release VIII stores from cells)
346
Pityriasis rosea
```
Itchy!
Herald patch
Christmas Tree Pattern
Common to have a URI prodrome
Etiology unknown
Not contagious
Lasts 2-12 weeks
```
347
Itchy rash on webs between fingers
Scabies
348
Palmar rash differential:
RMSF, Syphillis, Kawasaki, enterovirus (HFMD)
349
Celiac Diseasec
Disease of small intestine
Associated with Type 1 DM
Fatigue, weight loss
Iron deficiency (low MCV, high RDW)
Dermatitis herpetiformis on extensor surfaces
DIAGNOSIS: IgA anti-tissue transglutaminase antibody, SI biopsy
350
Dandy-Walker anomaly
hydrocephalus, cystic expansion of the 4th ventricle
more info
351
Chiari Malformation
hydrocephalus, protrusion of the structures of the posterior fossa through the foramen magnum
more info
352
Truncus arteriosus
???
353
Varicella treatment
acyclovir (herpes too!) (it's nephrotoxic though)
354
Membranous Glomerulonephritis
associated with HepB (asians)
See edema
hypoalbuminemia
elevated urine protein
355
Most common complication of having sickle cell trait (not disease)?
Hematuria.
Isosthenuria (impairment in concentrating ability) is also common and can present as nocturia or polyuria.
356
Inherited caused of unconjugated hyperbilirubinemia (2)
Inherited deficiencies of UDP-glucuronyl transferase in Crigler-Najjar (total deficiency) and Gilbert's Syndrome (mild def.)
357
What is erythroblastosis?
Hemolytic disease of the newborn (HDN) is a blood disorder in a fetus or newborn infant in which the mothers antibodies attack the fetal blood cells. See a +Coombs test, unconjugated hyperbili, and anemia.
358
Live vaccines (6)
```
Viral:
MMR
flu nasal spray
varicella (chicken pox)
oral polio vaccine
rotavirus
```
```
Bacterial:
BCG vaccine (TB vaccine)
```
359
Isolation, poor language skills, and inattentiveness are all signs of what?
Undetected hearing impairment
360
How does bringing knees to the chest improve some cardiac conditions?
It increases systemic vascular resistance, resulting in increased blood flow from the right ventricle to the pulmonary circulation. Helps for TOF babies.
361
Coffee-grinds vomit, Radiooppaque tablets on abdominal CT, abdominal pain
Iron poisoning. Leads to metabolic acidosis (low bicarb). Treat with chelator DEFEROXAMINE, the antidote for iron poisoning.
362
How do you treat a lithium toxicity?
Hemodialysis
363
How do you treat lead poisoning?
chelate with calcium EDTA.
| Can use oral succimer if poisoning is mild.
364
Reasons for high ammonia (normal <20um)
```
Liver failure
Hypovolemic shock
CHF
Infections (Hepatiti, HSV)
Medications (valproic acid)
IEMs (urea cycle, FAO, organic acidemias)
```
365
4 etiologies of high anion gap metabolic acidosis
Lactic acidosis
| Ketoacidosis
366
Normal values for AST and ALT?
AST: 6-40
ALT: 3.5-5
367
Acid-fast stains for...
Mycobacteria (including TB)
368
Causes of pancytopenia
```
Aplastic anemia
Myelodysplastic syndrome
Leukemia
Systemic lupus erythematosus
viral infections (HIV most common)
Pernicious anemia
Medication
Radiation
Gaucher's disease
```
369
Gaucher's disease
Gaucher disease is a rare genetic disorder in which a person lacks an enzyme called glucocerebrosidase.
370
Leishmania
a disease caused by protozoan parasites of the genus Leishmania and transmitted by the bite of certain species of the sand fly in tropical locations.
Features:
cutaneous ulceration
enlarged spleen, liver
pancytopenia
371
Normal electrolyte ranges?
| | /
____________________________
| | \
372
What is Ecthyma gangrenosum?
An infection of the skin typically caused by Pseudomonas aeruginosa. It is often seen in immunocompromised patients such as those with neutropenia.
373
What is Ranitidine (Zantac)?
H2 receptor antagonist forGERD treatment
374
Medication for absence epilepsy
Ethosuximide (or valproic acid)
375
Infantile spasms/West syndrome treatment
ACTH injections (or valproic acid)
376
Autoimmune disease with anti-GBM antibodies to collagen IV, see renal (nephritis) and pulmonary findings
Goodpasture's Syndrome
377
Croup treatment
Dexamethasone (steroid)
| Racemic epi if needed
378
Treatment for yeast/candida?
Nystatin (antifungal agent)
379
What is the Potter Sequence?
urinary tract abnormality --> anuria/oliguria en utero --> oligohydraminos --> pulm hypoplasia, flat facies, and limb deformities.
380
Risk factors for cholecystitis (3)
SS anemia
Hereditary spherocytosis
Obesity
381
This vitamin deficiency is common in rural/developing countries due to malnourishment and fat malabsorption (CF, cholestatic liver disease). It has benefits to children with measles.
What is the vitamin and what are some other manifestations of deficiency?
Vitamin A.
```
Other manifestations:
night blindness
complete blindness
xerophthalmia (can't prod. tears)
immunodeficiency (recurrent infections)
```
382
Elevated RDW (>20%)
Iron deficiency anemia
383
What an angiofibroma?
Juvenile angiofibromas are benign nasal growths that are capable of eroding (incl. bone erosion) and locally invading surrounding tissues.
Presents with:
nasal obstruction
visible nasal mass
frequent nosebleeds
384
What does the nitroblue tetrazolium test look for?
Chronic granulomatous disease--Defect of phagocytic cells due to dysfunction of the NADPH oxidase enzyme complex. The test is ABNORMAL when it comes back negative.
385
WHat kind of immunodeficiency come with DiGeorge Syndrome?
T cell deficiency.
386
Breast feeding contraindications
```
maternal:
active TB
HIV infection
Herpetic breast lesions
Perinatal Varicella infection
chemotherapy
drug and alcohol abuse
c
fetal galactosemia
```
387
What do you do for varicella prophylaxis?
Varicella vaccine! within 5 days of exposure.
388
What is Ehlers-Danlos Syndrome?
```
Disorder of type 3 or 5 collagen structure.
Hyper molbile joints
easy bruising
poor wound healing
soft, velvety, hyperelastic skin
```
389
```
Diagnose:
vomiting
headaches
bitemporal hemianopsia
CALCIFIED lesion above sella turcica
```
increased ICP, most likely a **Craniopharyngioma**
resembles tooth bud enamel
supresellar location, presses on optic chiasm
associated with DI
growth retardation
treatment: surgery
390
Many sinopulmonary and GI infections, episodic diarrhea, transfusion anaphylaxis--all point to what?
IgA deficiency (have antibodies against IgA).
391
5p deletion with microcephaly, hypotonic, short stature, and cat-like cry...
Cri-du-chat Syndrome
392
How to diagnose and treat pertussis
nasopharyngeal cultures (4 weeks).
Treat with macrolide abx (azithromycin, clarithromycin)
393
How quickly should you correct hyponatremia?
10-12 mEq in the first day, so about .5 mEq per hour. Also, hypertonic saline (3%) should primarily be given to those who are seizing from hyponatremia.
394
What anomalies are ear pits and ear tags associated with?
Renal anomalies
395
Noonan Syndrome
```
mutation in PTPN11 on Chr. 12
hypertelorism (wide set eyes)
downslanting eyes/palpebral fissures
webbed neck
pulmonary stenosis
coagulation defects and easy bruising
low set ears
shield chest
developmental delay
```
396
What is Motrin actually?
Ibuprofen
397
What is Tylenol actually?
Acetaminophen
398
Marfan's Syndrome + thromboembolic events = ?
Classic homoctstinuria (cystathionine synthase deficiency). Treat with high doses of vitamin B6.
399
Sx: perianal itching, vulvovaginitis, worse itching at night when they lay eggs. Diagnose with Scotch tape test.
Organism?
Treatment?
Pinworms/enterobius vermicularis
| Treated with ALBENDAZOLE!
400
What is propanolol?
Beta blocker to treat HTN, anxiety and panic.
