Pediatrics Flashcards
APGAR Scoring
Appearance Pulse Grimace Activity Respiration
1point on APGAR means
A- Pale blue extremities P- <100/min G- grimace on suction/stimulation A- some flexion R- irregular, shallow gasps
2 points on APGAR means:
A- pink P- > 100 G- cry on stimulation A- flexion that resists extension R- robust crying
What does the one minute APGAR Score tell?
How labor was tolerated by the newborn
5 mins APGAR score tells …….
Response of the newborn to resuscitation
Moro response on a LGA (Large gestational age) newborn: R arm remains limply adducted, medially rotated.
Diagnosis?
Erb- Duchenne palsy (C5-C6)
Moro response on a LGA (Large gestational age) newborn: R arm remains limply adducted, medially rotated.
Management?
- Physical therapy
- Surgery for severe cases
Paralysis of newborn hand +/- Horner syndrome
Klumpke palsy (C7 - C8 + T1)
Palpate the clavicle on LGA newborn- Crepitus and discontinuity on L.
Diagnosis?
Clavicular fracture
In a newborn: edema crosses suture lines
Caput succedaneum
In a newborn:
Fluctuance does not cross suture lines.
Diagnosis?
Cephalohematoma
Lacy, reticulated vascular pattern over most of body when baby is cooled; improves over first month; abnormal if persists
Diagnosis?
Cutis marmorata
White papules, retention of keratin and sebum in hair follicles
Diagnosis?
Milia
Inflammatory papules and pustules
Diagnosis?
Neonatal acne
Pale, pink vascular macules; found in nuchal area, glabella, eyelids; usually disappears
Diagnosis?
Salmon patch (Nevus simplex)
Blue to slate-gray macules; seen on presacral, back, posterior thighs; > in on white infants; arrested melanocytes; usually fade over first few years;
Diagnosis? And differential?
Mongolian spots
Child abuse
Firm, yellow-white papules/pustules with erythematous base; peaks on second day of life; contains eosinophils; benign
Erythema toxicum neonatorum
Vomiting,seizures, developmental delay (1st few months), intellectual disability + fair hair, eyes, skin, musty smell
Diagnosis?
Phenylketonuria
Jaundice, vomiting, HSM, FTT, poor feeding+ liver dysfunction, susceptibility to infection/ sepsis (E.coli), cataracts
Most common deficiency?
Galactose-1-phosphate uridyl transferase (GALT)
Diseases screened in new born: 8
Phenylketonuria Galactosemia Cystic fibrosis Hypothyroidism 21- hydroxylase deficiency Tyrosinemia Hb SS Hb C
Thick, yellow/white oily scale on inflammatory base
Management ?
Gently clean with mild shampoo (seborrheic dermatitis)
Area of alopecia, with orange-colored modular skin
Management?
Remove before adolescence (nevus sebaceous)
5 advantages of breast milk
- maternal-infant bonding
- premixed, right temperature and concentration
- less diarrhea, intestinal hemorrhage, chronic illnesses later in life
- maternal weight loss and faster return of uterus to preconception size
- decreased allergies accompanied to formula fed
Holds head steadily; rolls from prime to supine; coos
Age?
4 months
Sits with support (tripod); unilateral reach; transfers object; recognizes that someone is a stranger
Age?
6 month
“Mama”, “dada”; crawls well; pulls to stand; immature pincer grasp;
Age?
9 months
50 words; 2-word sentences; follows 2-step commands; parallel play
Age?
24 months
Alternates feet going up the stairs; pedals tricycle; >/= 250 words; 3 word sentences; group play;
Age?
3 years
Hops and skips; dresses completely; knows colors; play cooperatively
Age?
4 years
Prints first name; asks what a word means; knows alphabet; skips alternating feet; abides by rules
Age?
5 years
Infant ______ birth weight by 6 months and _______ by 1 year
Doubles
Triples
Bone age=chronological age
Normal cause
Ideal
Genetic (familial) short stature
Bone age= chronological age
Abnormal cause?
- Genetic
- Chromosomal
Bone age < chronological age
Normal cause?
Constitutional delay
Bone age < chronological age
Abnormal cause?
- chronic systemic disease
- endocrine related
Bone age > chronological age
Normal cause?
Obesity (tall)
Familiar tall stature
Bone age > chronological age
Abnormal cause?
- precocious puberty
- congenital adrenal hyperplasia
- hyperthyroidism
Immunization at birth?
HepB
Immunization at 2,4 and 6 months?
HepB, DTaP, Hib, PCV13, IPV
Immunization at 6 months and then yearly?
Influenza
Immunization at 12 months ?
MMR, Varicella HepA
Immunization at 4-6 yrs (before start of school)?
DTaP, IPV, MMR, Varicella
Immunization at 12 years?
Tdap, HPV, meningococcal meningitis
Upward slanting palpebral fissures; inner epicanthal folds; single palmar crease; hypotonia; hearing loss
Diagnosis?
Down syndrome (trisomy 21)
Most common cardiac anomalies seen in Down syndrome
Endocardial cushion deficiency
Most common cancer seen in Down syndrome
Acute lymphocytic leukemia
AML if in the first 3 yrs of life
Low-set, malformed ears, microcephaly, micrognathia, prominent occiput, clenched hands, rocker bottom feet, omphalocele.
Diagnosis?
Edwards syndrome (trisomy 18)
Holoprosencephaly, microcephaly, microphthalmia, cutis aplasia, single umbilical artery, severe cleft lip, palate, or both
Diagnosis?
Patau syndrome (trisomy 13)
Hypogonadism and hypogenitaliam; long limbs; decreased IQ; most common findings manifest at puberty
Diagnosis?
Klinefelter syndrome (XXY)
Large ears, dysmorphic facial features, large jaw, long face, intellectual disability, large testes
Diagnosis?
Fragile X syndrome
Macrosomia, macroglossia, pancreatic B cell hyperplasia, omphalocele
Diagnosis?
Beckwith- Wiedemann syndrome
Cafe at lait spots, soft tumors on nerves, seizures, CNS tumors, bone lesions
Neurofibromatosis
Micrognathia, retroglossia, cleft soft palate, associated with FAS or Edward’s
Diagnosis?
Pierre Robin sequence
Obesity, small puffy hands and feet, small genitalia, intellectual disability, hypothalamic-pituitary dysfunction
Diagnosis?
Angelman syndrome (happy puppet syndrome)
Short palpebral fissure, maxillary hypoplasia, short and smooth philtrum-vermillion border, symmetric IUGR
Fetal Alcohol Syndrome
When is hyperbilirubinemia pathologic?
- appears on 1st day of life
- Bili > 19.5mg/do (term baby)
- Bili increases >5mg/dL/day
- direct bili > 2 mg/dL (any time)
- persists beyond 2nd week of life
Newborn- Hypoxemia then hypercarbia, respiratory acidosis, ground-glass appearance, air bronchogram
Management?
Oxygen then intubation then exogenous surfactant (respiratory distress syndrome)
Tachypnea after birth, slow absorption of fetal lung fluid
Best test and what are the findings?
CXR
Air trapping, fluid in fissures, perihilar streaking
(Transient tachypnea of the new born
Pulmonary hypoplasia, respiratory distress, scaphoid abdomen, bowel sounds in chest
Best test to confirm diagnosis?
CXR (diaphragmatic hernia)
Infant (<28 days) with fever > 100.4 F =? Until proven otherwise
Sepsis
Most common pathogens associated with sepsis
Group B strep
E.coli
Listeria monocytogenes
Empiric treatment when suspicious of sepsis in a newborn
Ampicillin + Gentamicin x 48
Sepsis in neonates + meningitis suspected?
Cefotaxime + ampicillin
TORCH meaning?
Toxoplasmosis Others - syphilis, varicella, HIV, parvovirus B19 Rubella CMV Herpes
Maculopapular rash (palms+ soles), snuffles, periostitis, chorioretinitis
Diagnosis?
Congenital syphilis
Maculopapular rash (palms+ soles), snuffles, periostitis, chorioretinitis
Treatment?
Penicillin
Hydrocephalus, intracranial calcifications, chorioretinitis in neonates
Diagnosis
Toxoplasmosis
Hydrocephalus, intracranial calcifications, chorioretinitis in neonates
Treatment?
Pyrimethamine, sulfadiazine, leucovorin
Hydrocephalus, intracranial calcifications, chorioretinitis in neonates
Prevention?
Mother should not change at little
Spiramycin to decrease transmission
Cataracts, deafness, cardiac defects (PDA, VSD, etc), extra medullary hematopoiesis (“blueberry muffin spots”)
Diagnosis?
Rubella
Microcephaly, IUGR, periventricular calcification, decrease platelets, petechiae
Diagnosis?
Cytomegalovirus
Microcephaly, IUGR, periventricular calcification, decrease platelets, petechiae
Treatment?
Ganciclovir
Limb hypoplasia, scars, cataracts, chorioretinitis, cortical atrophy
Diagnosis?
Congenital varicella
Fever, seizure, bulging anterior fontanelle; mom has vesicles on perineum
Diagnosis ?
Congenital herpes simplex
1-3 DOL (day of Life): red conjunctivae, tearing
Diagnosis?
Chemical conjunctivitis
3-5 DOL: purulent conjunctivitis leading to corneal ulceration
Diagnosis?
Gonococcal conjunctivitis
3-5 DOL: purulent conjunctivitis leading to corneal ulceration
Treatment ?
IM ceftriaxone + saline irrigation
Gonococcal conjunctivitis
7-14 DOL: red tarsal conjunctivitis with mucoid discharge, lid swelling
Diagnosis?
Chlamydia conjunctivitis
7-14 DOL: red tarsal conjunctivitis with mucoid discharge, lid swelling
Treatment?
Erythromycin + saline irrigation
Early, systolic, soft, vibratory/ musical, heard best at lower sternum
Still’s murmur
Continuous murmur heard best in anterior neck which disappears when jugular vein is compressed
Diagnosis?
Venous hum
When is a murmur not benign
Diastolic murmur, holosystolic murmur, > II/VI, harsh or blowing quality
Common cyanotic heart diseases? 5 T’s
Tetralogy of fallot Truncus arteriosus Tricuspid atresia Transposition of Great vessels Total anomalous pulmonary venous return
CXR: “egg on a string” appearance
Loud, single S2
PDA closes leads to cyanosis, tachypnea
Diagnosis?
Transposition of the Great arteries
Components of tetralogy of fallot
- pulmonary stenosis and infundibular stenosis (obstructed RV outflow)
- VSD
- Overriding aorta (overrides VSD)
- Right ventricular hypertrophy
Bipolar mom gives birth to infant with holosystolic murmur c/w tricuspid regurgitation
Diagnosis?
Ebstein anomaly
Newborn: continuous machine-like murmur, bounding pulses, wide pulse pressure
Diagnosis?
Patent ductus arteriosus
Infant with normal brachial pulses, weak femoral pulses, rib notching on CXR
Diagnosis?
Coarctation of the aorta
ASD: what do you hear on heart auscultation?
Loud S1, fixed Split S2
VSD: what do you hear on heart auscultation?
Diagnosis?
Holosystolic murmur among the LLSB with a palpable thrill
When is surgery indicated for VSD?
FTT in first year, pulmonary hypertension, pulmonary blood flow X2 systemic blood flow
15 y.o. athlete suddenly collapses on the football field
Management?
BB or CCB
6 y.o. female w/severe joint pain (elbows, wrists), fever x 1 week, sore throat 1 month ago, exam reveals new murmur
Diagnosis?
Acute rheumatic fever
Newborn Caucasian mail fails to pass meconium in first 24 hrs of life. Diagnostic test?
Sweat chloride test
Respiratory infection in cystic fibrosis:
Early-
Then-
Later-
Nontypeable H. influenza and S. aureus
Then colonization with P. aeruginosa
Later colonization with Burkholderia cepacia
Failure of umbilical ring closure in an infants
Diagnosis?
Umbilical hernia
Management of small umbilical hernia in an infant
Watch and wait- resolves in 1-2 years without treatment
When should surgery be considered for umbilical hernia?
- enlarging after 1-2 years
- symptoms (strangulation, incarceration)
- persistent after age 4 years
Defect in abdominal wall with bowel outside laterally with no sac; high maternal AFP; not usually associated with other disorders
Diagnosis?
Gastroschisis
Bowel midline outside the abdominal cavity in a sac; associated with other disorders
Diagnosis?
Omphalocele
4- weeks old non-bilious vomiting + palpable “olive”
Diagnosis?
Pyloric stenosis
2- wk old w/ bilious vomiting (polyhydramnios in pregnancy)
Diagnosis?
Intestinal atresia (annular pancreas)
Premature infant presents with bloody stools, apnea, lethargy and abdominal distention (after perforation)
Diagnosis?
Necrotizing enterocolitis
Pathognomic finding on X-ray for necrotizing enterocolitis is
Pneumatosis intestinalis ( air in bowel wall)
Premature infant presents with bloody stools, apnea, lethargy and abdominal distention (after perforation)
Management?
Cessation of feeds
Gut decompression
Systemic antibiotics
Supportive care
6-12 months old; colicky abdominal pain, currant jelly stool, sausage shaped palpable mass in RUQ
Diagnosis and treatment?
Intussusception
Barium or air enema, surgery if recurs
Bright red blood per rectum (BRBPR) +/- RUQ (mimics appendicitis)
Diagnostic test?
Technetium scan
Meckel’s diveriticulum
12 years old Male presents with edema,
HTN, hematuria with colored urine, reports diagnosis of impetigo 1 month ago. U/A shows dysmorphic RBCs + RBC casts
Renal biopsy reveals?
Lumpy-bumpy Ig and complement deposits on GBM
12 y.o. Male wi th bloody urine; U /A shows dysmorphic RBCs +RBC casts + just started runny nose, sore throat and cough yesterday
IgA nephropathy (Berger’s disease)
12 y.o. Male wi th bloody urine; U /A shows dysmorphic RBCs +RBC casts + has hemoptysis
Diagnosis?
Anti-GBM Antibody disease (Goodpasture’s)
12 y.o. Male wi th bloody urine; U /A shows dysmorphic RBCs +RBC casts + bilateral sensorineural hearing loss + ocular abnormality
Alport’s syndrome (hereditary nephritis)
What mutation is seen in Alport syndrome?
Genes encoding for type IV collagen
Kidney stones <5mm
Management?
Hydrate- most pass spontaneously
Stones 5mm- 2cm
Management?
Extracorporeal shock wave therapy)
Stones > 2 cm
Endoscopic or open surgical removal
> 3.5g protein/ 24hrs., hypoalbunimemia, edema, hyperlipidemia (fatty/ waxy casts)
Most common cause in children?
Minimal change disease
Electron microscopy of minimal change disease shows?
Foot process fusion (effacement)
Newborn Male, no palpable testes
Diagnosis?
Cryptorchidism
Newborn Male, no palpable testes
Next best test?
Ultrasound
Newborn Male, ventral urethral opening
Hypospadias
Newborn Male, ventral urethral opening
Contraindication
Circumcision
Newborn w/ ambiguous genitalia at 1 mo with vomiting, decreased Na, increased potassium, acidosis
Diagnosis?
Congenital adrenal hyperplasia
Newborn w/ ambiguous genitalia at 1 mo with vomiting, decreased Na, increased potassium, acidosis
What is deficient?
21-hydroxylase
Newborn w/ ambiguous genitalia at 1 mo with vomiting, decreased Na, increased potassium, acidosis
Definitive test
17- OH progesterone before and after ACTH bolus
Newborn w/ ambiguous genitalia at 1 mo with vomiting, decreased Na, increased potassium, acidosis
Treatment?
Hydrocortisone + fludrocortisone
Teenage boy, Acute pain, swelling, tenderness, testicle in transverse lie
Diagnostic test?
Doppler color flow ultrasound
Acute painful scrotal swelling in sexually active male;
Epididymitis
Palpable, hard mass, does not trans-illuminate, usually painless
Testicular tumors
Pharmacotherapy for primary enuresis?
Oral desmopressin (DDAVP)
9.5 lb. newborn, jittery, ruddy with heart murmur
Infant of a diabetic mother
Metabolic abnormalities seen in infants of a diabetic mother
Hypoglycemia, hypocalcemia, hypomagnesemia
12 y.o. female w/ vomiting, wt. loss over the last month despite polyphagia and polydipsia + polyuria
Most likely diagnosis?
Type 1 DM
First line management of diabetic keto acidosis
IVF + insulin drip
Diagnosis of DM 1 made by symptoms + elevated glucose
FBG ?
2hrs OGTT ?
Random BG ?
FBG > 126
OGTT > 200 after 2hrs
RBG > 200
Renal threshold for glucose reabsorption =?
180mg/dL
In DM 1, there are antibodies against ________, __________, _________ or ________
Insulin, glutamine acid decarboxylase, tyrosine phosphatase or zinc transporter 8
8 y.o. African American boy w/ fever, chest pain, SOB, wheezing, hypoxemia
Acute chest syndrome (sickle cell disease)
In sickle cell disease blood smear shows what?
Sickled cells, increased reticulocytes, Howell jolly bodies (nuclear remnants), targets cells (Hb SC)
How do you monitor for risk of stroke in a patient with sickle cell disease?
Transcranial doppler
Progressive decrease in Hb over first 2-3 months (to Hb of 9-11g/dL)
Physiologic anemia of infancy
18 mo- old female “picky eater” who drinks a lot of cow’s milk, Hb = 6.5 g/dL, Hct = 20%, MCV = 65fL
Iron deficiency anemia
Iron study changes seen in iron deficiency anemia
Decrease ferritin, increase TIBC
Short stature, craniofacial deformities, upper extremities defects (triphalangeal thumbs), profound anemia by 2-6 mos is consistent with
Blackfan-Diamond
18 mos old w/ pancytopenia, cafe-au-lait spots, microcephaly and absent thumbs
Most likely diagnosis?
Fanconi anemia
2 y.o. w/ hyperactivity, impaired growth, abdominal pain, constipation, microcytic, hypochromic anemia, basophilic stippling of RBCs
Most likely diagnosis?
Lead poisoning
Treatment of lead poisoning
Chelation
4 y.o. w/petechiae, purpura, and excessive bleeding from oral mucosa
Immune thrombocytopenic purpura
15 y.o. female w/ heavy menses, petechiae but normal plts, increased bleeding time, prolonged PTT
Most likely diagnosis?
von Willebrand disease
Treatment of von willebrand disease
DDAVP, vWF replacement
1 week old born at home with bleeding from umbilical stump
Likely diagnosis?
Vit K deficiency
Treatment for vit K deficiency
Fresh frozen plasma
3 y.o. w/bloody diarrhea Nd decreased urination;5 days ago; fever & vomiting after a family picnic; patient appears pale and lethargic
Hemolytic uremic syndrome
Etiologies of hemolytic uremic syndrome
E.coli 0157:H7, shigella, salmonella, campylobacter, viruses, drugs
6 y.o. Male with palpable purpura, abdominal pain and knee pain
Most likely diagnosis?
IgA vasculitis Henoch-Schonlein purpura)
Panhypopituitarism, growth failure, visual field defects
Tumor?
Craniopharyngioma
3 y.o. with abdominal mass noted by mother + HTN
Most likely ________
Nephroblastoma (wilms tumor)
3 y.o. with periorbital ecchymosis, proptosis, bluish skin nodules and tender abdominal mass
Most likely diagnosis?
Neuroblastoma
4 y.o. female with limp and rash; exam: fever, HSM, petechiae
Most likely diagnosis?
Acute lymphoblastic leukemia
16 y.o. male w/ fever, wt loss, drenching sweats, nontender cervical LNs
Hodgkin lymphoma
7 y.o. female with persistent cough and mediastinal mass…
Suspect?
Non-hodgkin lymphoma
3 y.o. girl with “grape-like masses” growing from her vagina, per mom
Suspect?
Rhabdomyosarcoma (botryoid variant)
X-ray:-Sclerotic destruction: “sunburst”
Osteogenic sarcoma
X-ray:-Lytic with laminar periosteal elevation: “onion skin”
Ewing sarcoma
X-ray:-small round central lucency with sclerotic margin
Osteoid osteoma
Cough, cough, coryza, conjunctivitis, high fever, koplik spots, morbilliform rash
Diagnosis?
Measles
Complications of measles (4)
Otitis media
Pneumonia
Encephalitis
Subacute sclerosing panencephalitis
Mild constitutional symptoms, forchheimer spots, posterior cervical and auricular nodes, rash spreads from face to trunk and extremities
Diagnosis ?
Rubella
Headache, fever, malaise, muscle pain, glandular swelling
Complications ?
Encephalitis
Orchitis
Pancreatitis
Low grade fever, malaise, URI symptoms, crops of papules, vesicles that crusts at the same time, central to peripheral
Diagnosis?
Varicella
Low grade fever, malaise, URI symptoms, crops of papules, vesicles that crusts at the same time, central to peripheral
Complications?
Superinfection Zoster Pneumonia Encephalitis Hepatitis Congenital varicella
Mild URI symptoms, slapped cheek -> trunk -> central clearing-lacey
Diagnosis?
Erythema infectiosum
Parvovirus B19
Complications of parvovirus B19 infection
Aplastic anemia
Dangerous for pregnant mothers
URI symptoms, abrupt onset, high fever then breaks -> macular rash on trunk and spreads to extremities
Diagnosis?
Roseola
Sore throat, exudative pharyngitis, strawberry tongue, fine maculopapular rash (like sandpaper, esp antecubital and in GUI make areas), pastia lines
Diagnosis?
Scarlet fever (streptococcal infection)
Complications of scarlet fever
Acute rheumatic fever
Glomerunephritis
Usually absent (maybe fussiness, low-grade fever); oral macule -> vesicle w/halo of erythema; macular, maculopapular or vesicular; hands, feet and buttocks common
Diagnosis?
Hand-foot-mouth disease (coxsackie-virus)
Treatment of Lyme disease?
Doxycycline
Treatment of carditis and meningitis complications of Lyme disease ?
Ceftriaxone
7 y.o. w/fever, headache, rash that started on extremities (lives in wooded area of N. Carolina)
Rocky Mountain spotted fever
3 y.o. with honey-colored crusted plaques on face
Impetigo
Treatment for impetigo
Topical mupirocin
10 y.o. male w/ multiple excoriations and linear burrows in webs of fingers, ++pruritis
Scabies
1 week old infant w/ conjunctivitis, blisters on skin, + Nikolsky’s sign
Staphylococcal scalded skin syndrome
2-12 mos; fever lethargy, irritability, anorexia, N/V, meningeal irritation (photophobia, neck & back pain, rigidity, brudzinski, kernig or bulging fontanelle in infant)
Meningitis
6 yo male fromPA returns from camping with fever, headache and rash
Most likely diagnosis?
Lyme disease (borrelia burgdorferi)
Treatment for Lyme disease?
Doxycycline
Treatment for Lyme disease in a child < 8y.o.
Amoxicillin
Treatment for carditis or meningitis complication of Lyme disease
Ceftriaxone
New born; cluck on Barlow; Positive ortolani
Management?
Pavlik harness (developmental dysplasia of hips)
5 y.o. with progressive limping (initially painless now anterior thigh pain)
Management?
Casting or orthoses, rest, exercises, surgery
Legg- Calve- Perthes disease
Obese 14y.o. with knee pain and decrease range of motion of hip
Management?
Surgical stabilization
Slipped Capital femoral epiphysis
Complications of slipped capital femoral epiphysis
Osteonecrosis
Chrondolysis
6 y.o. male had URI 2 wks ago, now: limp, pain in groin and knee; non-toxic appearing kid, X-rays of hip and knee WNL, slight increase in ESR
Diagnosis?
Transient synovitis
10 y.o. basketball player with knee pain and swelling
Most likely …
Osgood-Schlatter disease
2-12 mos; fever lethargy, irritability, anorexia, N/V, meningeal irritation (photophobia, neck & back pain, rigidity, brudzinski, kernig or bulging fontanelle in infant)
Etiologies?
S. pneumonia, N. meningitidis, HiB
Meningitis in 0-2 months most likely organisms
GBS, E.coli, L. monocytogenes
Meningitis in 0-2 months, empirical antibiotics
Ampicillin + cefotaxime
Meningitis in 2-3 months; most likely organisms
GBS, E. coli, L. monocytogenes +
Some S. pneumoniae +
very little H. influenza type B
Meningitis in 2-3 months; empiric antibiotics
Ampicillin + caefotaxime/
Ceftriaxone + vancomycin (assume resistant S. pneumoniae)
Meningitis 3 months- 2 years; most likely organisms
S. pneumoniae + N. meningitides
3 months - 2 years with meningitis; empiric antibiotics
Vancomycin + cefotaxime/ ceftriaxone
2-18 years with meningitis; most likely organisms
N. meningitides +
2-18 years with meningitis; empiric antibiotics
Vancomycin + cefotaxime / ceftriaxone
19 y.o. male with roommate at college with bacterial meningitis and a petechial rash. What does this patient need?
Rifampin po x 2days
Treatment for otitis media
Amoxicillin
8 y.o. female with sore throat and fever over 1 day; exam: enlarged, erythematous tonsils with exudate, cervical lymphadenopathy
Acute pharyngitis
Complications of acute streptococcal pharyngitis
- Acute rheumatic fever
- Post streptococcal glomerulonephritis
- Reactive arthritis
Treatment of acute pharyngitis in a patient with allergy to penicillin
Erythromycin
Sore throat, “hot potato “ muffled voice, trismus, ipsilateral ear pain, uvula displacement
Most likely _____
Peritonsillar abscess
16 y.o. with sore throat, fever, fatigue, generalized adenopathy, splenomegaly
Infectious mononucleosis
3 y.o. with barking cough, hoarseness, inspiratory strider
Croup
Management of different severity of croup
Mild- sg dose dexamethasone
Mod- nebulized epinephrine + corticosteroid
Severe- nebulized epi + corticosteroid + admission
2 y.o. with fever of 102 F, tugging on ear; TM bulging and red
Most sensitive test
Pneumatic insufflation
Otitis media
Otitis media treatment in a patient with penicillin allergy
Azithromycin
Most common cause of croup?
Parainfluenza
3 y.o. with fever, tripod position, drooling
Epiglottis
3 y.o. with fever, tripod position, drooling
First line management
Intubation
9-mo-old infant w/runny nose, cough, wheezing, ↑work of breathing; RR = 60, + retractions, O2sat = 91%
Most common cause
RSV
Bronchiolitis
Prevention of bronchiolitis for premature patients, those with chronic lung disease or congenital heart disease
Palivizumab
6-mo-old infant w/coughing spells (loud inspir whoops) f/b vomiting
Confirm diagnosis by ________
Nasopharyngeal cx or PCR
Whooping cough; Bordetella pertussis
Treatment of cystitis in young children
amox, TMP/SMX, nitrofurantoin
Treatment for pyelonephritis in children
3rdgen cephalosporin 10-14 days
2 y.o. male with multiple episodes of otitis media, pneumonia and diarrhea. On exam, no tonsils seen
X-linked (Briton) Agammaglobulinemia (XLA)
17 y.o. female with ↓IgG, IgM, IgE, IgA but normal B cells
Common Variable Immunodeficiency (CVID)
2 y.o. female with giardiasis, PMH: pneumonia and UTI; Anaphylaxis with blood transfusion
IgA deficiency
3-day-old male with hypertelorism, thin upper lip, small mandible, notched ears presents with seizures and cyanosis
DiGeorge syndrome (thymic hypoplasia)
Infant with severe infections, no thymus, no tonsils, severe lymphopenia
Severe Combined Immunodeficiency (SCID)
18-mo-old male with severe eczema, petechiae, recurrent ear infxns.
Wiskott-Aldrich Syndrome
2 y.o. male with 4thepisode of otitis media; mom remembers his umbilical cord stump separated around 2 mos.
Leukocyte Adhesion Deficiency
3 y.o. male, recurrent swollen, infected LNs in groin
Test of choice
Dihydrorhodamine 123
Chronic granulomatous disease
7 y.o. girl w/L wrist and R knee pain (off and on x 3 mos.), pain is worst in morning (improves through day); R knee has effusion and ↓ROM
Juvenile Idiopathic Arthritis (JIA) (synovitis of peripheral joints)
Juvenile Idiopathic Arthritis (JIA) (synovitis of peripheral joints) treatment options
NSAIDs, methotrexate, azathioprine, cyclophosphamide, biologicals, steroids
2 y.o. female w/2-wk h/o fevers to 102°F, conjunctivitis, very red tongue, cracked lips, desquamating rash, swollen hands and feet
Kawasaki disease
Treatment for Kawasaki disease?
IVIG +high dose aspirin
Complication of Kawasaki disease
Aneurysm
feeding difficulty, choking,stridor, apnea, vocal cord paralysis,UE spasticity + spina bifida oculta/ meningocele/ myelomeningocele
Next step?
Head Ct : look for Arnold Chiari malformation
Presents: head circum > 95%ile, bulging fontanelle, distended scalp veins, “setting sun” eyes, ↑DTRs, vomiting
Hydrocephalus
↑ing head size, prominent occiput, spasticity, hyperreflexia, ataxia delayed motor development; Diagnosis?
Dandy-Walker malformation (agenesis or hypoplasia of cerebellar vermis, cystic dilation of 4thventricle, enlarged post fossa)
Treatment for simple partial seizures
Phenytoin
18-mo-old male w/first time tonic-clonic seizure lasting 3 mins. He has had a viral illness with temp up to 103.8°F
Febrile seizure
Management for febrile seizure
Acetaminophen
10 y.o. female with falling grades in school and many episodes of ”staring into space” with eye flickering; EEG shows 3 Hz spike
Absence (petit mal )seizure
Management for absence seizure
Ethosuximide *drug of choice) or valproic acid
6-mo-old w/symmetric contractions of neck, trunk and extremities; EEG: hypsarrhythmia (asynchronous, chaotic spike-wave pattern)
Infantile spasm
6-mo-old w/symmetric contractions of neck, trunk and extremities; EEG: hypsarrhythmia (asynchronous, chaotic spike-wave pattern)
Treatment?
ACTH, prednisone, +/- anticonvulsant
10 y.o. male w/ataxia, explosive speech that is difficult to understand, kyphoscoliosis
Friedreich ataxia
10 y.o. male w/ataxia, explosive speech that is difficult to understand, kyphoscoliosis
Most common cause of death
HOCM—>
CHF, arrhythmia, death
13 y.o. male w/dystonia, tremors, Kayser-Fleischer rings in his eyes, and recent behavioral changes
Best test?
Serum ceruloplasmin
13 y.o. male w/dystonia, tremors, Kayser-Fleischer rings in his eyes, and recent behavioral changes
Diagnosis
Wilson disease
13 y.o. male w/dystonia, tremors, Kayser-Fleischer rings in his eyes, and recent behavioral changes
Treatment?
Chelation with penicillinamine
10-mo-old Jewish male w/seizures, hypotonia, cherry-red macula
Tay-Sachs disease
What is deficient in Tay-Sachs disease
Beta-hexosaminidase A
9 y.o. male w/choreoathetosis and spasticity + h/o renal calculi
Lesch-Nyhan disease
Newborn lying in frog-leg position w/subdiaphragmatic retractions, absent DTRs
Spinal Muscle Atrophy (SMA) (AR degenerative dz of motor units)
11 y.o. male w/clumsiness (dropping items) and frequent falls; LEs are “stork like” + pes cavus
Charcot-Marie-Tooth (HMSN I)
3 y.o. male w/clumsiness and big calves;
Duchenne Muscular Dystrophy
Cause of becoming wheel bound in duchenne muscular dystrophy
Calf pseudohypertrophy (deposits of fat and collagen) + thigh muscle wasting
What is seen on muscle biopsy for Duchenne muscular dystrophy
Abnormal or absent dystrophin
Initial best for diagnosis of duchenne muscular dystrophy
CPK (Increased)
Presents: facial wasting (“inverted V” upper lip, thin cheeks, scalloped temporalis, narrow head, high arched palate) + hypotonia (progressive wasting: distal →proximal) + poor speech, difficulty swallowing, EOM weakness, slow GI emptying + heart block + endocrine probs + half w/intellectual disability
Myotonic dystrophy (CTG trinucleotide expansion)
Disorders that are associated with omphalocele (2)
Beckwidth-Weidemann syndrome, trisomy 18
