Pediatrics Flashcards
(264 cards)
1
Q
When does neonatal conjunctivitis from Chlamydial infections occur? Treatment?
A
5-14 days after delivery?
Treatment: ORal erythromycin
2
Q
Linear lesions with pruritus and erythematous streaks with edema and vesicles after camping?
A
Poison Ivy- Contact Dermatitis
3
Q
Complications of hemophilia A and B
A
Hemophilic arhropathy -> iron/hemosiderin deposition leading to sunovitis and fibrosis
4
Q
Todd paralysis
A
self-limited, focal weakness that occurs after focal or generalized seiure; presents in the postictal period with partial or compelte hemiplegia involving ipsilateral arm
5
Q
Herpetic Gingitcostomatitis: Cause, Signs
A
Cause: HSV1
Signs: Fever, Pharngitis, Erythematous gingiva, Clusters of small vescicles on anerior oropharynx
6
Q
Signs of Diamond-Blackfan syndrome?
A
Macrocytic anemia, low reticulocyte count, congenital anomalies
Average age of onset is 3 months
7
Q
Painful vescibles with “punched out” erosions and hemorrhagic crusting superimposed on a child with atopic dermatitis?
A
HSV
8
Q
Legg-calve-perthes disease
A
Average onset 4-10, incisious onset, antalgic gait (less time on one side), long-term pain
9
Q
Most common bacterial infection in children with CF?
A
S. Aureus, especially with concurrent influenza.
10
Q
Ehlers-Danlos syndrome
A
scoliosis, joint laxity, skin hyperelasticity
11
Q
Signs on leukocyte adhesion deficiency?
A
Recurrent skin and mucosal infections, no pus, poor wound healing
12
Q
Most common cause of sepsis in sickle cell patients?
A
S. pneumoniae
13
Q
X-linked agammaglobulemia?
Alternative name?
A
Low B-cells, low Ig levels
AKA: Bruton’s agammaglobulinemia
14
Q
First step in the evaluation of a neonate with emiesis?
A
Cessation of enteral feeds, NG tube decompression, IV fluids, XRay to rule out pneumoperitoneum
15
Q
When should you suspect tricuspid atresia?
A
Cyanotic infant with left axis deviation, small or absent R waves,
16
Q
Signs of vitamin D deficiency in an infant?
A
Delayed fontanel closure, enlarged skull, costochondral joints (achitic rosary), long-bone joint widening, genu varum (bowed legs)
17
Q
Children under 8 years of age should be treated for erythema hronicum migrans from lyme disease with?
Over 8?
A
Under 8: Amoxicillin
Over 8: Doxycycline
18
Q
What is spondylolisthesis? Signs?
A
Developmental disporder characterized by forward slip of vertebrae (L5 over S1)
Signs: Chronic back pain, neurological dysfunction, palpable step-off
19
Q
Job syndrome?
A
Normal levels of B-cells, Ig, except with hyper IgE
20
Q
Acute management of a sickle cell patient with signs of stroke?
A
Exchange transfusion, dilutes amount of sickled cells in blood
21
Q
Developmental milestones at 12 months?
A
Stands well, walks first steps, 2-finger pincer, says first words, separation anxiety, comes when called
22
Q
Early adolescent obese male that presents with hip or knee pain of insidious onset that causes limping? Treatment?
A
Slipped capital femoral epiphysis
x: surgical pinning
23
Q
Fibrosarcoma
A
Malignant spindle cell neplasm found in patiens 30-60. Painful mass. Osteolytic lesion whose margins can be well-defined or ragged and moth-eaten
24
Q
Congenitial CMV infections signs? Treatment?
A
Growth restriction, microcephaly, periventirular calcification, hepatosplenomegaly, thrombocytopenia
Treatment: Valganciclovir
25
Osteosarcoma
Findings: Soft tissue mass, "sunburst pattern", periosteal elevation (Codman traingle)
26
Beckwith-Wiedemann syndrome: Cause? Clincal Manifestations? Complications? Surveillance?
_Cause_: Desregulation of imprinted gene expression on 11p15
_Manifestations:_ Fetal meacrosomia, rapid growth, macroglossia, hemihyperplasia, medial abdominal wall defects
_Complications_: Wilms tumor, hepatoblastoma
_Surveillance:_ Serum AFP, abdominal/renal US
27
Signs of sepsis?
Fever, chills, hypotension, leukocytosis, bandemia
28
How to diagnose Laryngomalacia?
Confirmation by flexible laryngoscopy for moderate-severe cases
29
VACTER
Vertebral, anal atresia, cardiac abnormalities, radial (skeletal) and renal
30
What findings of a pediatric burn would suggest child abuse?
Sparing flexor surfaces
31
Diagnostic test for Chronic Granulomatous disease?
Neutrophil function testing
- Dihydrohodamine 123 test
- nitroblue tetrazolium test
32
Heinz bodies
Aggregates of denatured hemogobin, seen in patients with hemolysis due to G6PD deficiency and thalassemia
33
Total anomalous pulmonary venous return
4 pulmonay veins fail to make the normal connection to the left atrium. Righ atrium received blood from both pulmonary and systemic venous system.
Signs: right venticle hypertrophy, and right axis deviation
34
Pyridoxine (B6) deficiency
Cheilosis, stomatitis, glossitis, irritability, confusion, depression
35
Acute Rheumatic fever: Major and minor signs
_Major_: Migratory Arthritis, Carditis, Subcutaneous nodules, Erythema marginatum, Sydenham Chorea
_Minor:_ Fever, arthralgias, Elevated ESR, CRP, Prolonged PR interval
36
condensed nuclear chromatin, small nucleoli with scant agranular cytoplasm
Lymphocytes
37
Herpangina: cause, signs
Cause: Coxachie A virus
Signs: Feverm pharyngitis, gray vesicles/ulcers on posterior oropharynx, hands, feet
38
Clincal signs of glucose-6-phosphatase deficiency?
Lab Findings?
Doll-like face, thin extremities, short stature, protuberant abdomen
_Lab findings_: Hypoglycemia, high blood uric acid, total cholesterol, triglymerides, lactic acid levels
39
What is the next step in management after capillary samples tested positive for increased lead?
Venous blood lead level
40
Tests used to diagnose DM type 1?
glytamic acid decarboxylase (GAD65), IGF-1, IGFBP
41
Aldolase B deficiency causes?
Fructose intolerance- vomiting, poor feeding, lethargy, serizures or enxephalopathy
42
Hemolytic uremic syndrome: Signs? Lab findings? Treatment?
_Signs_: Diarrhea, lethargy, irritability, pallor, bruising or petechiae, oliguria, edema
_Lab findings_: Hemolytic anemia, thrombocytopenia, Incresed hematuria, proteinuria, casts, increased bilirubin
_Treatment_: Fluid/electrolyte management, blood transfusion, dialysis
43
What is a small for gestations age newborns at risk for developing?
Hypoxia, perinatal asphyxia, meconium aspiration, hypoglycemia, hypothermia, hypocalcemia, and polycythemia (due to hypoxia)
44
What congenital heart defects are present in patients with Down syndrome? Presentation?
Complete atrioventricular septal defect
Signs: Loud S2 d/t pulmonary hypertension
Systolic ejection murmur (increased flow across pulmonary valve from left to right shunt)
Holosystolic murmur of VSD (soft or absent if defect is large)
45
When does alloimmune hemolytic disease occur?
"A" and "B" offspring of "O" mothers
46
What cardiovascular abnormalities are in turner's syndrome?
Bicuspid aortic valve
47
Findings suggestive of hypertrophic adenosis?
Postnasal drip, elongated facial heatures, mucopurulent nasal discharge, loud mouth breathing
48
VSD murmur description?
Pansystolic murmur hear loudest at the left lower sternal border and a diastolic rumble at the apex due to increase flow across mitral valve
49
Hereditary spherocytosis
Inc. mean corpuscular hemoglobin concentration
Spherocytes on peripheral smear
Negatove coombs test
Increased osmotic gragility on acidiied glyceral test
Abnormal eosin-5-meleimide binding test
50
Child has a desire to kill his mother and must recite a prayer 10-12 times to overcome it. Diagnosis? Treatment?
_Diagnosis_: Obsessive-compulsive disorder- can be obsessive (symmetry), or compulsive (must hurt mother)
_Treatment_: First-line: SSRI (Fluoxetine) or CBT
Second-line: Clomupramine and antipsychotic augmentation
Third-line (refractory): Deep-brain stimulation
51
Severe combined innumodeficiency: Signs
lymphopenia, recurrent infections, and failure to thrive,
ADA deficiency
52
Defect in Prader-Willi syndrome
Deletion of the paternal copy of chromosome 15q11-q13
53
Most effective form of emergency comtraception?
Copper IUD
54
Tetralogy of Fallot murmur?
Harsh systolic ejection murmur over the left upper sternal border, single S2
55
Workup for a newborn with bilious vomiting without a bowel movement?
Abdominal Xray -\> contrast enema
56
Methylphenidate-Use?
ADHD
57
High blast count with strongly positive periodic acid schiff (PAS) reaction, in a 2-10 year old?
Lymphoblastic leukemia
58
What type of vaccination prevents S. pneumoniae?
Conjugate capsular polysaccharide
59
Kallman Syndrome
X-linked recessive disorder of migration of fetal gonaotropin-releasing hormoes and olfactory neurons.
_Clincal Signs_: short stature, delayed or absent puberty, normal genotype and internal reproductive organs, anosmia/hyposmia
60
Red flags for type 1 DM
Polyuria, polydipsia, enuresis (increased urinary incontinence)
61
When should gonads be removed in complete androgen insensitivity?
After puberty, the benefits of gonad-stimulated puberty outweigh the risk of malignancy
62
Alternative name for laryngotracheititis?
Croup
63
Signs of increased intracranial pressure?
Morning vomiting and noctural headaches
64
Erythema marginatum of Rheumatic fever
65
What disorders are commonly associated with mitral valve prolapse?
Connective tissue disorders (Marfan, Ehlers-Danlos sundrome, osteogenesis imperfecta, etc)
66
Infants born to diabetic mothers are at risk for?
Hypoglycemia, hypocalcemia, hyperbilirubinemia, polycythemia
Cardiomyopathy, GI atresia, urinary malformations, hyaline membrane disease
67
Most common cause of abnormal uterine bleeding in adolescents?
Immature hypothalamic-pituitary-ovarian axis -\> anovulation cycles
68
Signs of pinworm infections?
Perianal puritus, especially at night, may extend into the volvula in pre-pubertal women
69
Cyanotic when stressed and asymptomatic at rest?
Tetralogy of Fallot
70
Chediak-Higashi syndrome
AR disorder characterized by oculocutaneous albinism and recurrent cutaneous infections
Staph. aureus and Strep pyogenese are most common
71
Components of DiGeorge's Syndrome?
Conotruncal cardiac defects
Abnormal Facies
Thymic aplasia/hypoplasia
Cleft palate
Hypocalcemia
(t-cell lymphopenia is present)t
72
Burton agammaglobulinemia- Lab findings
Normal T-cells, absent B cells, low serum immunoglobulin, absent lymphoid tissue
73
When is a murmur benign?
Normal activity levels, early of mid-systolic, Grade I or II, low-putched muscicle or squeaky tone at LLSB (still's murmur) or high-pitched at LUSB (pulmonary flow murmur)
74
Signs of botulisism?
Bilateral bulbar palsies (ptosis, sluggish pupillary response to light, poor sucking), constipation, drooling, and summetric descending flaccid paralysis
75
Caput seccedaneum
diffuse, sometimes ecchymotic swelling of the scape. Usually involved the portion of the head presenting during vertex delivery. May extend across midline and suture lines
76
Most common cause of orbital cellulitis?
Bacterial sinusitis
77
OsGood-Schlatters Disease; traction apophysitis
78
What are pink stains or "brick dust" in neonatal diapers?
Uric Acid crystals
79
Seborrheic dermatitis:
Erthematous plques and/or yellow, greasy scales
80
What are turner's syndrome at increased risk for?
Osteoporosis- they lack protective estrogen production -\> increased bone reabsorption
81
Cause of intussusception in children younger than 2? Older?
_\< 2_: recent viral illness or rotavirus vaccination
_\>2_: Meckel's diverticulum, HSP, Celiac disease, intestinal tumor, polyps
82
Non-classical congenital adrenal hyperplasia (late-onset)
Premature adrenarche/pubarche, sever cystic acne (resistent to treatment), accelerated linear grwoth, advanced bone age
83
In a child less than 24 months, what should occur after the treatment of her first febrile UTI?
Renal and bladder ultrasound
84
Signs of fetal alcohol syndome
Small head, small palebrl fissures (eyes), smooth philtrum, thin vermilion border (lip)
85
What gross motor milestones should occur at 18 months?
Run, kicking a ball
86
Adolescent male
Benign bone tumor, osteoid osteoma
**Pain is relieved by NSAIDS**
87
Treatment for Impetigo?
Oral cephalexin (widespread)
mupirocin (topical, localized)
88
Fetal exposure to cocaine
Jitteriness, excessive sucking, hyperactive moro reflex
89
What should all sexually active women under 24 be screened for?
Chlamydia trachomatis and neisseria gonorrhoeae
90
Risks of maternal diabetes?
Macrosomic getus, shoulder dystocia (crepitus, asymmetric moro reflex), clavicle fracture, brachial nerve palsies, perinatal asphyxia, neonatal hypoxic encephalopathy, polycythemia
91
Heroin intoxication in a fetus, signs?
Interuterine growth retadation, irritability, high-pitched cry, poor sleeping, tremors, sweating, sneezing, diarrhea
92
What type of vasculitis is Henoch-Schlnlein purpura? Complications?
Immunoglobulin A-mediated
Complications: renal involvement; microscopic or macroscopic hematuria, red cel casts, mild to moderate proteinuria, with normal-slightly elevated serum creatinine
\*\*Can lead to nephrotic syndrome, HTN, acute renal failure
93
Heart defect associated with Edwards Syndrome? Murmur description?
VSD
Holosystolic murmur that is heard beast at the lower left sternal border
94
Purpose of the CH50 test?
Determines total complement levels
95
Risk of gonad-derived cancer in turners syndrome compared to general population?
10-30 times higher (15-30%). Gonad development should be closely monitored and an earlier gonadectomy could be required.
96
Clinical manifestations of Prader-Willi? Common complications?
Poor suck and feeding problems in infancy, compulsive binge-eating and obesity-related problems
_Complications:_ Sleep Apnea, DM type II
97
What can cause flaccid paralysis? How?
Infant botulism- ingestion of C. botulinum spores from dust
Foodborne- C. botulinum toxin from food
Guillain-Barre syndrome- Autoimmune peripheral nerve demyelination
98
Tinea Capitis, dermatophyte infection
Tx: Oral terbinefine, friseofulvin, itraconazole, fluconazole
99
Measles vs Rubella
Measles: High fever (\>104), cephalocaudal rash over multiple days , cervical adenopathy
Rubella: maculopapular cephocaudal rash over 24 hours (sparing palms and soles), polyarthralgia
100
Patient with meningitis and petechial rash?
Neisseria meningtides
101
What solutions should be used to resusitate an infant that is hypovolemia, but hypernatremia?
0.9% NS
102
Niacin (b3) deficiency
Pellegra (dermatitis, diarrhea, delusions, glossitis)
103
Riboflavin deficiency signs:
Angular choelosis, stomatitis, glossitis, normocytic anemia, seborheic dermatitis
104
Treatment for migraines in pediatric populations?
Acetaminophen or NSAID and supportive mangement (dark room, etc)
105
Clinical features of medulloblastomas?
Ataxia and truncal instability
106
Alternative name for Wilms tumor?
Nephroblastoma
107
Henoch-Schonlein purpura: Signs?
transient migratory arthritis (lower extremities) and rash (puritic)
108
What is the generic name for the plan B pill? MoA
Levonorgestrel
_MoA:_ Delays ovulation
109
Primary dusmenorrhea? Treatment?
Lower abomdinal cramping with menses in the absense of other pathology
\*\*pain during the first few days of menses is cause ny uterine contractions triggered by prostaglandins
_Treatment:_ NSAIDS
110
Wiskott-Aldrich syndrome: Clincal manifestations? Defect? Problem?
Eczema, recurrent infections, thrombocytopenia
_Defect_: WASP protein
_Conequence:_ Impaired cytoskeleton changes in leukocyte, platelet
111
How to treat a patient with a "barky" cough?
Racemic epinephrine and corticosteroids
112
What signs should suggest possible child abuse
Abrupts onset of mood changes, bedwetting, and/or academic difficulties
113
Erythrocte CD55 and CD59 are present in which disease?
Paroxysmal noctural hemoglobinuria
114
What diseases can lead to a vitamin K deficiency?
Cystic fibrosis, IBS, Celiac, biliary atresia, frequent Abx use, malnutrition
115
Signs on Gonadotropin-independent precocious puberty?
Boy under 9 with severe facial ance, advanced pubic and axillary hair, normal testicular volume
116
Signs of refeeding syndrome? Cause?
Arrhythmia, congestive heart failure, seizures, wenicke encephalopathy
Cause: Increased insulin secretion -\> decreased phosphorus, magnesium, potassium, thiamine, with increased water and sodium
117
Galactosemia
"mouse-like" odor, hyperbilirubin, elevated liver function tests, low glucose, cataracts, ascities
Increased risk for E. Coli sepsis
118
Signs of hemophilia?
Hemarthrosis and Deep tissue hematomas
Increased aPTT
119
2 days fever and facial swelling with delayed vaccination schedule? Most common complication?
Mumps
_Common complication:_ Aseptic meningitis, orchitis, parotitis
120
Howell Jolly bodies?
nucleated remnant of red blood cells
121
What are the catecholamines?
homovanilic acid and vanillylmandelic acid
122
Aeur rods are found in which type of cancer/
Acute myelocytic leukemia`
123
What factors increase the risk for intraventricular hemorrhages?
Preterm and loss birth weight
124
How to diagnose intraventicular hemorrhage?
transfontanel ultrasound
125
Myotonic musculodystrophy-Onset? Inheritance? Comorbidies? Prognosis?
126
What increases the murmur n Hypertrphic cardiomyopathy? Decreases?
_Increases:_ Decreased preload: Valsava, abrupt standing, nitroglycerine
_Decreases_: Increased afterload/preload: Sustained hand grip, squatting, passive leg raises
127
In which population in the cyclic vomiting syndrome the highest?
Children of parents who have a history of migraines
128
abdominal pain, palpable mass, jaundice
Biliary cyst
129
47 XXY
Klinefelter syndome, male phenotype with small testicles.
130
What is the most common event prior to a seizure? During?
_Prior_: Aura
_During_: Tongue Laceration
131
What type of CT should be ordered for head trauma?
Non-contrast
132
What substance maintains the PDA? Closes it?
Maintains: Prostoglandin E2
Closes: Indomethicine
133
Clincal manifestations of medulloblastoma?
increased intracranial pressure, trucal and gait ataxia
134
How to assess pulmonary function in a guilllain-barre patient?
Spirometry, peak flow could be used as an alternative
135
What prophylaxis should be used for cat bites in children?
Amoxicillin/Clavulanate due to activity again pasteurella multocida
136
Primary vs.Secondary dysmenorrhea
Primary- normal physical examination
Secondary - abnormal physical examination
137
Signs of congenital CMV?
pericentricular calcifications (microcephaly, if severe)
138
What infections are common in sickle cell patient?
H influenza, N meningitidis, S. pneumoniae
Al
139
Signs of Turner's Syndrome?
Narrow, high arched palate, low hairline, webbed neck , broad chest, coarctation of the aorta, bicuspid aortic valve, horse-shoe kidney, streak ovaries, amenorrhea, infertility
140
Signs of Tetralogy of Fellot?
Tet spells-sudden hypoemia and cyanosis during exertion or agitation
141
What are risk facors of Henoch-Schonlein purpura?
Gastrointestinal hemorrhage or intussusception: Cause by bowel edema and hemorrhage
142
Common complication of individuals with sickle cell trait?
Hematuria
143
Cephalohematoma
subperiosteal hemorrhage, not visable until several hours after birth
144
Triad of brain abscesses
Headaches (noctural and/or morning), focal neurologic changes, fever
145
Maple syrup urine disease
dystonia, poor feeding, lethargy, vomiting, ketouria
146
What is the treatment for mild lead levels?
Moderate lead levels?
Severe lead levels?
Mild: No medication, retest in \<1 month (5-44mcg/dL)
Moderate: DMSA (Meso-2,3-demercapto-succinic), (45-69mcg/dL)
Severe: Dimercaprol (British anti-lewisite) plus EDTA (\>69mcg/dL)
147
Aplastic anemia vs aplastic crisis?
_Aplastic anemia_: pancytopenia due to bone marrow failure
_Aplastic crisis_: Chronic anemia with compensatory reticulocytosis
148
Signs of syphilis in a newborn?
Profuse rhinorrhea, desquamating skin rash, abnormal long-bone radiographs (metaphyseal lucencies)
149
How should a urine sample be collected in infants with diapers?
Urethral catheterization, urinalysis, urine culture
150
Mental retardation, pronounced startle reflex, serizures, hyperacusis, loss of vision, macrocephaly
Tay sachs disease, beta-hexosaminidase A
151
Cause of chronic nasal obstruction refractory to conventional treatment?
Adenoid hypertrophy
152
How to follow-up an incidental urine proteinuria in an adolescent?
Repeat dipstick testing on two subsequent occaisions to determine orthostatic, persistent, or transient proteinuria
153
Alternative name for strawberry hemoangiomas?
Superficial infantile hemangioma
154
Phenttoin exposure in-utero
Fetal hydantoin syndrome- nail and digit hypoplasia, dysmorphic facies, mental retardation
155
Celiac disease risk factors?
First-degree relative
Autoimmune thyroiditis
Type 1 DM
Down syndrome
Selective IgA deficiency
156
What is the squirt sign?
Explosive gas and stool associated with Hirsprung Disease
157
Biliary atresia
Obstructive jaundice, acholic stools, presents early in infancy
158
Inability to extend the beck and widened prevertebral space suggests?
Retropharyngeal abscess
159
Gold standard for confirming muscular dystrophies?
Genetic analysis
160
Cough, coryza, conjunctivitis with subsequent exanthema?
Measles
161
Why are menstral cycles in pubertal girls irregular?
Immaturity of the hypothalamic-pituitary-honadal axis -\> decreased gonadotropin secretion
162
Mongolian spots, beling flat, blue-grey patches
Fade spontaneously, reassure parents
163
recurrent sinusitis, point of maximal impulse displaced to the left, bronchiectasis?
Primary ciliary dyskinesia (Kartagener syndrome,
164
How do you confirm the type of leukemia?
Bone marrow biospy
165
Meconium obstruction at th level of the ileum and narrow, underdeveloped colon?
Cystic fibrosis
166
Tinea Corporis
167
Ornithine transcarbamylase deficiency
vomiting and poor feeding, seizures, increased ammonia, altered mental status
168
Ewing Sarcoma
"onion skin", "moth eaten", extension into soft tissue
169
Selective mutism
Refusal to speak in a specific social situation, but engaged in normal communications where she feels comfortabe.
Diagnosis required 1 month or more in situations where they are required to talk (school), despite speaking in other situations
170
Characteristics of oppositional defiant disorder?
Angry/urrutale mood and argumentative/defiant behavior towards authority figures
171
Diagnosis?
Eczema Herpeticum-complication of atopic dermatitis
172
Short statute, primary amenorrhea, abscent thelarche
Turner's Syndrome
173
Treatment for Tinea Corporis?
First line (localized): Topical (clotrimazole, terbinafine)
Second-line (generalized): Oral antifungals (terbinafine, griseofulvin)
174
Cafe au lait spots, precocious pubert, multiple bone defects (polyostotic fibrous dysplasia)?
McCune Albright syndrome, remember the 3 P's: Precocious puberty, pigmentation, polyostotic fibrous dysplasia
175
Neimann-Pick vs. Tay sachs
Neimann-pick: Sphingomyelinase deficiency with hyporeflexia/areflexia and hepatosplenomegaly
Tay Sachs: beta-hexosaminidase A with hyperreflexia without hepatosplenomegaly
176
Whem should PEEP be employed in a newborn? Chest Compressions?
Heart rate less than 100, irregular breathing
Chest compressions: Less than 60bpm
177
Treatment for vaicella zoster exposure in an individual without complete immunity?
Postexposure prophylais with VAV vaccines (if within preceeding 5 days)
If unable to receive live vaccine: varicella immunoglobulin (includes individuals less than 1 year of age)
178
Signs of Meckel's diverticulum? Diagnosis? Treatment?
Asymptomatic incidental finding, painless hematochezie, intussusception, intestinal obstruction, volvulus
Diagnosis: Technetium-99m pertechnetalte scan
Treatment: Surgey for symptomatic patients
179
Difference between iron deficiency anemia and thalassemia?
_Iron deficiency_: Increaed RDW, decreased MCW, RBC #
_A-thalassemia_: Normal RDW, RBC #; decreased MCV, normal hemoglobin electrophoresis
_B-thalassemia_: Normal RDW, RBC #; decreased MCV, increased hemoglobin A2 with electrophoresis
180
What is a patient with recurrent infections and congenital heart disease at risk for?
Brain abscess
181
Edema, hypoalbuminemia, markedly elevated urine protein in a teen with Hep. B? Pathogenesis
Membranous nephropathy (nephrotic syndrome)
_Pathogenesis:_ Deposition of HbeAb and its corresponding antibody in the glomeruli
182
How to granulosa cell tumors present in children?
Post-menopausal woman?
Diagnostic findings?
_Children:_ Precocious puberty, Large adnexal mass
_Post-menopausal:_ bleeding/endometrial hyperplasia
_Diagnostic findings_: Inc. estrogen,
Pelvic US: ovarian mass, thickened endometrium
183
Signs of hypothyroidism? Lab?
Decreased activity, horse cry, jaundice
\*However, majority are asymptomatic at birth
Lab: Increased TSH, decreased T4
184
Clincal manifestation of Edwards Syndrome?
Trisomy 18
_Signs_: Closed fists, with overlapping fingers, micrognathia, prominent occiput, rocker-bottom feet, severe intellectual disability
185
Early onset puberty iwth high basal LH? Next step?
Gonadotropin-dependent precocius puberty
Next step: Brain MRI wtih contrast
186
Signs of HIV in infancy?
Failure to thrive, chronic diarrhea, lymphadenopathy, pneumocystis pneumonia
187
Breath-holding spells
Types?
Episodes of apnea precipitated by frustration, anger, pain.
**Types**
_Cyanotic_: crying followed by breath-holding, cyanosis and loss of consciousness
_Pallid:_ Minor trauma followed by breath-holding, pallor, diaphoresis, and loss of consciousness
188
Signs of breastfeeding faliure jaundice?
1st week of life,
Filaure of transition to yellowish or green stools (inadequate milk ingestion), decreased wet diapers
189
Cyanosis that is aggrevated by feeding and improved by crying
Choanal Atresia
190
Urinary in ontinence in children over 5?
_Workup_: Urinalysis, urological imaging
_Management_: Avoid caffeine, void regularly, drink fluids early, reward system, enuresis alarm (1st line after behavioral, pharmocotherapy (desmopressin, tricyclic antidepressants)
191
192
What causes the trendelenburg sign?
Weakness of the gluteus medius and gluteus minimus muscles, where are innervated by the superior gluteal nerve
193
What type of rash spares the palms and soles? What type of precaution should be used for this infection?
Measles, should use airborne precaution
194
ill-appearing neonate with bilious emesis and poor apetite?
malrotation with mid-gut volvulus
195
What is the defect in duchenne and becker muscular dystrophy?
Deletion of dystrophin on chromosome Xp21
196
What complications are CF patients at risk for?
Infertility (95% of men, 20% of womeN)
Pancreatitis (~10%)
197
Clincal features of pineal gland masses?
Limited upward gaze, upper eyelid retraction, puils non-reactive to light, reactive to accomidation
198
Signs of ADHD?
Inattentive, hyperactive/impulsive symptoms, prior to age 12
199
Marfan syndrome vs Homocystinurea
_Marfan:_ AD, normal intellect, Aortic root dilation,
upward lens dislocation
_Homocystinurea:_ AR, intellectual disability, thrombosis, downward lens dislocation, megaloblastic anemia, fair complexion
200
Extraintestinal signs of Celiac disease?
Short stature, weight loss, iron deficiency anemia, dermatitis herpetiformis
201
Risk factors for ARDS in a newborn?
Prematurity, maternal diabetes, male sex, perinatal asphyxia, c-secton without labor
202
Clincal features of Epiglottitis?
Abrupt onset Distress ("tripod", stridor), dysphagia, drooling, thumbprint sign on Xray
203
What can cause isolated adrenarche? Clincal manifestations? Lab findings?
Adrenal angrogen secretion in obese individuals
_Signs_: oily hair/skin, acne, pubic hair, axillar hair
_Lab findings_: Increased DHEA-S, low testosterone
204
How do you management Autism spectrum disorders?
Early diagnosis and intervention (speech, behavioral), adjunct pharmacotherapy for psychiatric comorbities
205
Clinincal manifestations of PNH?
hemolytic anemia, cytopenias, and hypercoagulability
206
Family history of sudden death, congenital sensorineural deafness, and QT interval of 600ms?
Jervell and Lange-Nielsen syndrome
207
Signs of cardiac temponade?
Distant heart sounds, distended jugular veins, (scalp veins in infants) and hypotension
208
Glomus tumor triad
severe intermittent pain, tenderness, sensitivity to touch
benign tumor of vascular system
209
When should formula supplementation be considered?
Greater than 7% loss of birth weight
210
Varicella immunization protocol
2 doses, 1st at 1 year, 2nd at 4 years
211
Signs of intraventricular hemorrhage?
Hypotension, cyanosis, seizures, focal neurological signs, bulding or tense hontamel, anpea, and bradycardia; however, may be asymptomatic
212
Most common cause of pneumonia in children?
Adults?
_Children_: S. aureus
_Adults_: Pseudomonas
213
Hemiparesis, aphasia after injury tot he posterior pharync
Cervical internal cartid artery
\*\*Can occur when falling with an object in mouth
214
Murmur in Tetralogy of Fallor?
crescendo-decreasendo systolic murmur with a single S2 in left upper sternal border
215
How to diagnose hereditary spherocytosis?
Osmoti gragility on acidified glycerol lysis test
216
Clinical manifestations of congenital diaphragmatic hernia? Managemtn?
Pulmonary hypertension, polyhydramnios, barrel-shaped chest, concave abdomen
_Management_: Emergency intubation with caution ventilation
217
Signs of hyper-IgM syndrome?
Normal B cells, decreased IgG/IgA, increased IgM
218
Cause of edema in a child with turners syndrome? Type of edema?
Dysgenesis of the lymphatic network
_Type:_ Non-pitting
219
Pyloric Stenosis: Onset age? Characteristics?
Onset: 3-5 weeks
Clinical presentatin: Nonbilious, hugry vomiter, poor weight gain, dehydrated, "olive-shaped" abdominal mass
220
Congenital Toxoplasmosis infection signs?
Macrocephaly, diffuse parenchymal calcifications
221
Signs on defective intracellular killing?
No neutrophilia, infections with catalse-positive organisms
222
Signs on Shigella infection?
Rapid onset, high fever, abdominal pain, watery diarrhea with mucous +/- blood, +/- seizures in children
223
Type 1 RTA?
_Defect_: Poor hydrogen secretion into urine
_pH_: \> 5.5
_Potassium_: Low-normal
_Cause:_ Genetic disorder, medication toxitiy, autoimmune
224
Trachoma? Signs?
Chlamydia trachomatis serotype A-C, major cause of blindness
Signs: follicular conjunctivitis and pannus (neovascularization) of the cornea
225
What are cholesteatoma? Cause? Complications?
retraction pocket in the tympanic membrane that fills with granulation tissue and skin debris
_Cause:_ Chronic middle ear infections or congenital in nature
_Complications:_ Hearing loss, cranial nerve palsies, vertigo, brain abscesses or meningitis
226
Infectious mononucleosis, signs?
Test?
Fever, Tonsillitis/pharyngitis, psterior or diffuse cervical lympadenopathy, significant fatigue, hepatosplenomegaly
_Test_: Heterophile antibody test
227
Focal Serizures
Originated from 1 cerebral hemisphere +/- loss of consciousness
Cannot respond to stimuli during episodes with confusion afterwards
\>2-3 minutes in length
228
Severe combined immunodeficiency- lab findings? Treatment?
Failure of T-cell development, b-cell dysfunction due to low T-cells, absence oflymphoid tissues
_Treatment_: Stem cell transplant
229
Causes of aplastic anemia?
Most common in children?
Acquired causes: Drugs (NSAIDS, sulfonamides)
Toxic chemicals (benzene, glue)
Idiopathic
Viral infecitions (HIV, EBV)
Immune Disorders
Thymoma
Most comon in children: Fanconi anemia (chromosomal break)
230
How to determine B lymphocytes from total lymphocytes?
Subtract T-lymphocytes from total lymphocytes
231
How to manage epiglottitis?
Endotracheal intubation, antibiotics
232
Croup vs Epiglottitis
_Croup_: Bark-like cough, stridor, hoarse voice
_Epiglottitis_: Unvaccinated children, sore throat, dysphagia, drooling, tripod position
233
Signs of pancreatic deficiency?
Bruises (vitamin K), Vitamin D deficiency, poor growth
234
Head tilt to one side with chin deviation away
Congenital muscular torticolis
235
Most common cause of otitis externa?
P. Aeruginosa, S. aureus
Tx: Fluoroquinolone
236
When should IV ceftriaxone be used to treat lyme disease?
Lyme meningitis and heart block
237
Diagnosis?
Treatment?
Diagnosis: Atopic dermatitis
Treatment: Emolients +/- steroid topicals
238
Medication treatment of abnormal uterine bleeding?
High-dose IV or oral estrogen
High-dose combined oral contraceptive pills
High-dose progestin pills
Tranexamic acid
239
Presentation of immune throbocytopenic purpura?
Thrombocytopenia and purpura, no anemia or renal invovlement
240
Tests used to screen for DM Type 2?
Hemoglobin A1c, fasting glucose, 2 hour oral glucose, 1,5-anhydroglucitol
241
First step in management for a harsh, holosystolic murmur best heart at the left lower sternal border?
Echocardiogram to evaluate ventricular septal defect
242
Signs on congenital rubella infection?
Sensorineural hearing loss
cataracts
PDA
243
Meniere's disease?
Ear condition associated with fluid in the inner ear that leads to hearing loss, vertigo, tinnitus
244
Drugs used to treat Tourette disorder?
Haloperidol, pirmozide, risperidone (preferred), clonidine, guanfacine
245
Signs of renal tubular acidosis?
Normal anion gap acidosis, failure to thrive, alkalotic urine without known etiology of acidosis, low serum bicarb and hypercalemia
246
Eczema, regurgitation/vomiting, and painless bloody stools
Milk protein-induced enterocolitis
247
What homronal imbalances causes polycystic ovary syndrome?
Excess LH secretion
248
Differences in blood pressure in coarctation of the aorta?
Hypertension in the upper body and hypotension in the lower
249
Signs of hydrocephalus
Tense and bulging fontanelle, prominent scalp veins, poor feeding, irritability, rapidly increasing head size
250
What is the management of an adolescent with suspected fibroadenoma?
Observation and reassurance (follow-up shortly after next menses
251
Neuroblastoma?
Most common extracranial tumor of childhood
- Adrenal gland or retroperitoneal ganglia
- Calcifications and hemorrhages are seen on xray/ct
252
How does a person with a volvulus present?
vomiting (often bilious)
253
Signs of orbital cellulitis?
Abrupt onset of fever, proptosis, restriction of extraocular movements and swollen, red eyelids
254
Signs of foreign body ingestion?
Difficulty swallowing, feeding refusal, vomiting
255
When should chilren be toilet trained?
_daytime_: around 3
_Nighttime_: Any time up until 5 is considered normal
256
How to manage a hydrocele in a newborn? 2 year old?
_Newborn_: Observation
_2 year old_: Surgical correction
\*\*Most spontaneously recover within 12 months
257
What is the first step in evaluating primary absence of menarche by age 15?
Pelvic US, to detect ovaries, uterus, and vagina
258
When legal guardians of a patient deny a life-saving procedure, how do you proceed?
Obtain a court order to force compliance
259
Adolescent with nasal obstruction, visible nasal mass, frequent nosebleeds, and bony erosions on hte back of the nose, suspect?
Angiofibroma
260
Lennox-Gastaut syndrome
Presents by age 5, intellectual disability and sever seizures of varying types. EEG demonstrated a slow spike-wave pattern
261
What are signs of turner syndrome?
Coarctation of aorta, broad chest, short stature, horse-shoe kidney, streak overies
262
Baby that is cyanotic when feeding, pink when crying? Associated abnormalities?
Choanal atresia
Associated: CHARGE: Coloboma, heart defects, retarded growth, GU abnormalities, ear abomalies, deafness
263
Big tongue, umbilical hernia?
hypothyroidism
264
Tetralogy of Fallot components?
VSD + RA hypertrophy + over riding aorta, pulmonary stenosis
