Pediatric Step 2 material
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| 418446352 | ASD endocardial cushion defects | Pediatric heart condition associated MC with Trisomy 21 | 0 | |
| 418446353 | PDA | Pediatric heart condition associated MC with congenital rubella. | 1 | |
| 418446354 | Coarctation of aorta (bicuspid aortic valve) | Pediatric heart condition associated MC with Turner's syndrome. | 2 | |
| 418446355 | Kawasaki disease | Congenital pediatric condition associated with coronary artery aneurysms. | 3 | |
| 418446356 | Neonatal lupus | Congenital pediatric condition associated with congenital heart block. | 4 | |
| 418446357 | Williams syndrome | ●"Elfin" facies ●Supravalvular aortic stenosis or other vascular anomalies [3] ●Hypertension ●Cognitive profile consisting of impaired cognition and development accompanied by a friendly, social personality ●Short stature ●Endocrine abnormalities including hypercalcemia, diabetes mellitus, and subclinical hypothyroidism ●Genitourinary abnormalities include congenital anomalies of the kidney and urinary tract, nephrolithiasis due to hypercalciuria, and dysfunctional voiding ●Auditory, dental, gastrointestinal, genitourinary, musculoskeletal, neurologic, ophthalmologic, and dermatologic abnormalities | 5 | |
| 418446358 | Ebstein's anomaly | Pediatric heart condition associated MC with maternal lithium use during pregnancy. | 6 | |
| 418446359 | Heart failure | Pediatric heart condition associated MC with neonatal thyrotoxicosis. | 7 | |
| 418446360 | Asymmetric septal hypertrophy and transposition of the great vessels | 2 Pediatric heart conditions associated MC with maternal diabetes. | 8 | |
| 418446361 | VSD | Harsh holosystolic murmur heard best at lower left sternal border in a pt with history of frequent respiratory infections, dyspnea, FTT, and CHF. echocardiogram is diagnostic. | 9 | |
| 418446362 | ASD | Fixed widely split S2. Surgical repair only indicated with a 2:1 ratio of pulmonary to systemic blood flow. | 10 | |
| 418446363 | sepsis, inborn errors of metabolism, ductal dependent congenital heart disease (usually left sided lesions), Congenital adrenal hyperplasia | 4 MC causes of shocklike state within the first few weeks of life. | 11 | |
| 418446364 | PGE1 | Drug given IV to maintain PDA for early onset cyanotic conditions. | 12 | |
| 725885579 | Transposition of the great vessles | MC cyanotic congenital heart lesion in the newborn. MC associated with diabetic mothers and digeorge syndrome. Managed at start with PGE1 or if surgery not applicable in first few days of life and PDA not remaining open then balloon atrial septostomy is temporary; surgical correction is definitive. | 13 | |
| 554948227 | tetrology of fallot | MC cyanotic congenital heart lesion in childhood. Risk factors include maternal PKU and digeorge syndrome. PE shows systolic ejection murmur on left upper sternal border, right ventricular heave, and single S2. history may suggest failure to thrive or mental status changes and children often squat. | 14 | |
| 136370986 | O2, propranolol, phenylephrine, knee chest position, fluids, morphine | Treatment of tetrology of fallot hypoxic spells acutely include these 6 steps. | 15 | |
| 756306845 | 2 months | lifts head/chest when prone (gross motor), tracks past midline (fine motor), alters to sound, coos (language), Recognizes parent, exhibits social smile (social/cognitive) | 16 | |
| 495345416 | 4-5 months | Rolls front to back, back to front (Gross motor), grasps rattle (fine motor), Laughs and squeals, orients to voice, begins to make consonant sounds (language), enjoys looking around, laughs (social/cognitive). | 17 | |
| 927520764 | 6 months | Sits unassisted (gross motor), transfers objects, demonstrates raking grasp (fine motor), babbles (language), demonstrates stranger anxiety (social/cognitive) | 18 | |
| 843744266 | 9-10 months(first word at 11 months) | Crawls, pulls to stand (gross motor), uses 3 finger pincer grasp (fine motor), says mama/dada nonspecifically (language), waves bye-bye, plays pat-a-cake (social/cognitive) | 19 | |
| 731585728 | 12 months | walks alone, throws objects (gross motor), uses 2-finger pincer grasp (fine motor), uses 1-3 words, follows 1-step commands (language), imitates actions, exhibits separation anxiety (social/cognitive) | 20 | |
| 864152612 | 2 years | Walks up/down steps, jumps (gross motor), builds tower of 6 blocks (fine motor), uses 2-word phrases (language), follows 2-step commands, removes clothes (social/cognitive) | 21 | |
| 808869873 | 3 years | Rides tricycle, climbs stairs with alternating feet (gross motor), copies a circle, uses utensils (fine motor), uses 3-word sentences (language), brushes teeth with help, washes/dries hands (social/cognitive). | 22 | |
| 62725979 | 4 years | Hops (gross motor), copies a cross (fine motor), knows colors and some numbers (language), exhibits cooperative play, plays board games (social/cognitive) | 23 | |
| 834268766 | 5 years | Skips, walks backward for long distances (gross motor), copies a triangle, ties shoelaces, knows left and right, prints letters (fine motor), uses 5-word sentences (language), exhibits domestic role playing, plays dress-up (social/cognitive) | 24 | |
| 152327126 | 8 years | precocious puberty is considered when secondary sexual maturation occurs in females before this age | 25 | |
| 619910918 | 9 years | Precocious puberty is considered when secondary sexual maturation occurs in males before this age. | 26 | |
| 335124260 | 13 years | Delayed puberty is considered when no breast development or pubic hair growth in females over this age. | 27 | |
| 137091493 | 14 years | Delayed puberty is considered when no testicular enlargment in boys by this age. | 28 | |
| 450603636 | Trisomy 18 (edwards' syndrome) | presents with severe mental retardation, rocker-bottom feet, low set ears, micrognathia, clenched hands (overlapping fourth and fifth digits), and a prominent occiput. associated with congenital heart disease, horseshoe kidney, and death usually occurs within 1 year of birth. | 29 | |
| 475852065 | Trisomy 13 (patau's syndrome) | presents with severe mental retardation, microphthalmia, microcephaly, cleft lip/palate, holoprosencephaly, punched out scalp lesions, polydactyly, and omphalocele. associated with congenital heart disease and death usually occurs within 1 year of birth. | 30 | |
| 92466273 | alpha galactosidase A (fabry's disease) | This enzyme deficiency leads to accumulation of ceramide trihexoside in the heart, brain, and kidneys. First sign is severe neuropathic limb pain along with joint swelling. skin may show angiokeratomas and telangiectasias. Findings include renal failure and an increased risk of stroke and MI (thromboembolic events). | 31 | |
| 947486446 | galactosylceramidase (krabbe's disease) | This enzyme deficiency leads to accumulation of galactocerebroside in brain characterized by progressive CNS degeneration, optic atrophy, spasticity, and death within the first 3 years of life. | 32 | |
| 123947313 | glucocerebrosidase (gaucher's disease) | This enzyme deficiency leads to accumulation of glucocerebroside in the brain, liver, spleen, and bone marrow. cells may have crinkled paper appearance and enlarged cytoplasm. May present with anemia and thrombocytopenia. Infantile version presents with early, rapid neurologic decline. Adult version is compatible with a normal life span and does not affect the brain. | 33 | |
| 767454568 | sphingomyelinase (niemann-pick disease) | This enzyme deficiency leads to accumulation of sphingomyelin cholesterol in reticuloendothelial and parenchymal cells and tissues. May present with cherry red spot and hepatosplenomegaly. Type A die by age 3. | 34 | |
| 316339505 | hexosamininidase (Tay-sachs disease) | This enzyme deficiency leads to accumulation of GM2 ganglioside with infants presenting with exagerated startle response, but most are normal until 3-6 months of age, then cherry-red spot. Death occurs by age 3. | 35 | |
| 683208804 | arylsulfatase A (Metachromatic leukodystrophy) | This enzyme deficiency leads to accumulation of sulfatide in brain, kidney, liver, and peripheral nerves. Demyelination leads to progressive ataxia and dementia. | 36 | |
| 820739061 | alpha-L-iduronidase (hurler's syndrome) | This enzyme deficiency leads to corneal clouding, mental retardation, and gargoylism. |  | 37 |
| 497673941 | Iduronate sulfatase (hunter's syndrome) | This enzyme deficiency leads to mild mental retardation and gargoylism and X-linked recessive. |  | 38 |
| 484207909 | Intussusception | The most common cause of bowel obstruction in the first 2 years of life (males > females) usually seen between 3 months and 3 years of age. Cause is unknown | 39 | |
| 86514146 | barium enema (or anorectal manometry in atypical presentation) | Initial diagnostic study for a presentation of failure to pass merconium in 48 hrs of birth accompanied by bilious vomiting and FTT although children with less severe forms present later in life with chronic constipation. PE rectal examination shows explosive discharge of stool. | 40 | |
| 487376738 | rectal biopsy | Definitive diagnostic study for hirschsprungs disease. | 41 | |
| 550002206 | pneumotosis intestinalis | intramural air bubbles representing gas produced by bacteria within the bowel wall and pathopneumonic for necrotizing entercolitis. |  | 42 |
| 874531904 | FTT | weight/height below 5 percentile or a drop in weight/height more than two percentile quadrants. | 43 | |
| 675511901 | Brutons congenital agammaglobinemia | X-linked recessive B-cell deficiency with symptoms beginning after 6 months of age when maternal IgG no longer is active. Characterized by life threatening infections from encapsulated pseudomonas, S. pneumoniae, and haemophilus. Absent tonsils and other lymphoid tissues provide clue while Ig levels are low and B cell counts are absent, T cell counts high. Tx with prophylactic antibiotics and IVIG. | 44 | |
| 825329062 | Common Variable immunodeficiency | Usually combined B and T cell defect with all Ig levels low (starting in 20-30's) though normal B-cell numbers (low plasma cells). symptoms usually present with increased pyogenic upper and lower respiratory infections and increased risk of lymphoma and autoimmune disease. Dx Quantitative Ig's and B/T cell subset counts. Tx IVIG | 45 | |
| 260490765 | IgA deficiency | Mild, but most common immunodeficiency. usually asymptomatic, but may develop recurrent respiratory or GI infections (Giardia). Anaphylactic transfusion reaction due to anti-IgA Ab's is common presentation. Qauntitative Ig's for Dx. Do not give IVIG due to increased risk of anti-IgA Ab's. | 46 | |
| 560161638 | Ataxia-telangiectasia (ATM gene) | Progressive difficulty with coordinating movements beginning in early childhood (<5 y/o). Ataxia, dyskinesia, dysmetria, chorea, myoclonus, neuropathy. Small clusters of enlarged blood vessels in eyes and skin. Develop chronic lung infections, increased risk of cancer (leukemia, lymphoma)Requires wheelchair by adolescence. Due to mutation in DNA repair. | 47 | |
| 541195569 | 6 months | Age at which infants may start to consume solid foods. | 48 | |
| 103044661 | 3 months | Strabismus is considered normal in infants this age or younger, but pathologic when older. | 49 | |
| 685564668 | Laryngotracheobronchitis (croup) | Most commonly occurs between ages 6 months to 6 years and has stridor associated with a barky cough, fever, rhinorrhea, and congestion | 50 | |
| 170767950 | Laryngomalacia | Stridor most severe at age 4-8 months with persistent stridor that worsens in supine position, but improves while in prone position. | 51 | |
| 498821856 | Foreign body aspiration | Strido has an acute onset and is associated wtih moderate-to-severe respiratory distress. | 52 | |
| 766357657 | Vascular ring | Stridor presents before age 1 year with a persistend nature that improves with neck extension and is associated with cardiac abnormalities (50%). | 53 | |
| 1000259947 | Rotavirus | The live attenuated virus vaccine has the following contraindications- anaphylaxis to vaccine ingredients, history of intussusception, history of uncorrected malformation of the GI tract (eg. meckel's diverticulum), severe combined immunodeficiency disease. small increased risk of causing intussusception and should not be given to those children with a hx of intusssusception. | 54 | |
| 564442010 | Henoch-schonlein purpura | presents with palpable purpura with normal platelet count and coagulation studies, arthritis/arthralgia, abdominal pain (GI bleeding and possibly intussusception), renal disease similar to IgA nephropathy, scrotal pain and swelling. |  | 55 |
| 890734473 | Lyme arthritis | Development of monoarticular arthritis after several months of migratory arthralgias and a trip to northeastern and upper midwestern states. aspiration of synovial fluid - Leukocytes = 20,000 Neutrophils= 50% Color = yellow Clarity = translucent Gram stain = Negative | 56 | |
| 221323536 | nonclassic CAH (congenital adrenal hyperplasia) | Presents with premature adrenarche/pubarche, severe cystic acne resistant to treatment, accelerated linear growth, and advanced bone age. There is also normal electrolytes. LH is low baseline and does not increase with GnRH stimulation. | 57 | |
| 99803377 | Leydig cell tumor | Presents with premature adrenarche/pubarche, severe cystic acne resistant to treatment, accelerated linear growth, and advanced bone age. There is also normal electrolytes and unilateral testicular mass. LH is low at baseline and does not increase with GnRH stimulation. | 58 | |
| 112995875 | Idiopathic precocious puberty | Characterized by premature activation of the hypothalamic-pituitary-gonadal axis accounting for 80% of gonadotropin-dependent precocious puberty occuring almost exclusively in girls. Basal LH usually elevated and increases with GnRH stimulation. | 59 | |
| 935943977 | Hyperhemolytic crisis | Rare complication of SCD characterized by sudden, severe anemia accompanied by a reticulocyte count of >1%. | 60 | |
| 274846404 | Vasoocclusive crisis | Complication of SCD characterized by acute onset of pain that may be precipitated by weather, dehydration, or infection. | 61 | |
| 207871979 | Splenectomy | Treatment necessary for a 7 y/o presenting with severe hypotensive shock, dramatic drop in hemoglobin concentration, persistent reticulocytosis, and rapidly enlarging spleen, 10-15% mortality, 50% chance of recurrence. | 62 | |
| 491717190 | Neonatal sepsis | 12 day old presenting with respiratory distress, vomiting, jaundice, temperature instability (including hypothermia), poor feeding, irritability or lethargy. | 63 | |
| 1563130580 | Staphylococcal scalded skin syndrome | 3 y/o presenting with red rash and blisters starting 8 hrs earlier. pt is febrile (102) and there is superficial flaccid bullae and an erythematous rash diffusely distributed over her body. Nikolsky sign positive. Her face seems edematous, and there is crusting around the mouth area. Dx? |  | 64 |
| 164143749 | Impetigo | Localized epidermal infection with blisters that are flaccid and rupture easily, leaving a honey-colored crust. Nikolsky sign negative. Lesions are most commonly asymptomatic. |  | 65 |
| 426333164 | Erysipelas | Bacterial infection of the dermis presenting with a well-demarcated, red, indurated, warm tnder plaque. occasionally with overlying bullae and vesicles; lymphangitic streaking away from the plaque. fever, chills, malaise are common. Group A strep is classic cause and the face and over extremities are common sites of involvement. |  | 66 |
| 1658088796 | Pneumothorax | Condition that must be ruled out in a patient with a past medical history of severe coughing paroxysms that resulted in subcutaneous emphysema = are leaks into chest wall into subcutaneous tissue to the high intraalveolar pressure provoked by cough. | 67 | |
| 601699661 | Cholesteatoma | Dx should be suspected with presentation of continued ear drainage for several weeks despite appropriate antibiotic therapy. congenital or acquired with acquired occurring secondary to chronic middle ear disease = retraction pocket in TM, which can fill with granulation tissue and skin debris. Complications include hearing loss, cranial nerve paslsies, vertigo, potential for brain abscesses or meningitis. |  | 68 |
| 1661836285 | Otosclerosis | Conductive hearing loss. |  | 69 |
| 253514322 | Hypertrophy of peyer patches | Lead point to severe, intermittent abdominal pain; drawing up legs with pain. episodes last 15-20 minutes. abdominal tenderness and palpable cylindical mass on right side of abdomen. Pt is 1.5 y/o and had diarrhea 1 wk prior. | 70 | |
| 126902066 | Meckel's diverticulum | Lead point to severe, intermittent abdominal pain; drawing up legs with pain. episodes last 15-20 minutes. abdominal tenderness and palpable cylindical mass on right side of abdomen. Pt is 6 y/o and had intussusception at age 2 that was successfully reduced by air enema. | 71 | |
| 294209877 | Myotonic dystrophy | AD expansion of CTG repeat in DMPK gene on 19q13/3. Age of onset 12-30 y/o; presents with facial weakness, hand grip myotonia, dysphagia; comorbidities of arrhythmias, cataracts, balding, testicular atrophy/infertility; prognosis is death from respiratory or heart failure depending on age of onset. | 72 | |
| 1163944351 | Trachoma | follicular conjuntivitis and pannus (neovascularization) formation in the cornea. concurrent infection occurs in the nasopharynx leading to nasal discharge. Dx made by giemsa stain of conjuntival scrapings. Tx with topical tetracycline or oral azithromycin. repeated infections can lead to scarring of the cornea. |  | 73 |
| 561026499 | Spondylolisthesis | 12 y/o presenting with slowly developing back pain and neurologic dysfunction, palpable step-off at the lubosacral area. |  | 74 |
| 567334898 | Conduct disorder | Disruptive behavioral patterns that violate basic social norms for at least one year. Includes aggression towards others or animals, property damage, theft, violations of social rules. Pt is <18 y/o. | 75 | |
| 359299805 | Antisocial personality disorder | Disruptive behavioral patterns that violate basic social norms for at least one year. Includes aggression towards others or animals, property damage, theft, violations of social rules. Pt is >18 y/o. | 76 | |
| 242307775 | B1 (thiamine) | Water soluble vitamin associated with Peripheral neuropathy, heart failure. Found in whole grains, meat, fortified cereal, nuts, legumes. | 77 | |
| 1384994776 | B2 (riboflavin) | Water soluble vitamin associated with angular cheilosis, stomatitis, glossitis, normocytic anemia, seborrheic dermatitis. found in dairy eggs, meat, green vegetables. | 78 | |
| 121479287 | B3 (niacin) | Water-soluble vitamin associated with dermatitis, diarrhea, delusions/dementia, glossitis. Found in meat, whole grains, legumes. | 79 | |
| 1870794684 | B6 (pyridoxine) | Water-soluble vitamin associated with cheilosis, stomatitis, glossitis, irrability, confusion, depression. Found in meat, whole grains, legumes, nuts. | 80 | |
| 861150133 | B9 (folate, folic acid) | Water-soluble vitamin associated with megaloblastic anemia and neural tube defects. Found in green leafy vegetables, fruit, meat, fortified cereal/grains. | 81 | |
| 1798996354 | B12 (cobalamin) | Water-soluble vitamin associated with megaloblastic anemia and neurologic deficits (confusion, paresthesias, ataxia). Found in meat and dairy. | 82 | |
| 1565097246 | C (ascorbic acid) | Water-soluble vitamin associated with punctate hemorrhage, gingivitis, corkscrew hair. Found in citrus fruits, strawberries, tomatoes, potatoes, broccoli. | 83 | |
| 1474899876 | Primary dysmenorrhea | Release of prostaglandins from endometrium causes uterine contractions. The PE is normal. Onset generally occurs during adolescence. | 84 | |
| 827704104 | Endometriosis | Bleeding from ectopic endometrium with PE demonstrating uterosacral nodularity and adnexal tenderness. | 85 | |
| 722336729 | adenomyosis | Bleeding from ectopic endometrium with PE demonstrating uterine tenderness and enlargment. | 86 | |
| 1157336708 | Pelvic infection | Bacterial infection and inflammatory response with PE demonstrating cervical motion tenderness, purulent cervical discharge | 87 | |
| 157702218 | Uterine leiomyomata | May be associated with heavy bleeding while PE demonstrates uterine contour irregularity. | 88 | |
| 1864774266 | Acute otitis media | Middle ear effusion plus acute eardrum inflammation. |  | 89 |
| 499290351 | Otitis media with effusion | Middle ear effusion without acute inflammation |  | 90 |
| 812364391 | Bullous myringitis | serous liquid-filled blisters on the tympanic membrane |  | 91 |
| 391527298 | Cerumen impaction | liquid or hard wax in auditory canal obstructing eardrum visualization. |  | 92 |
| 213898994 | Hemotympanum | Pulple or red eardrum +/- bulging |  | 93 |
| 897931273 | otitis externa | Pain with tragal traction, erythematous and swollen external auditory canal +/- otorrhea. |  | 94 |
| 1955334317 | Herpangina (cocksackie A virus) | 1-mm gray vesicles on the tonsillar pillars and posterior oropharynx that progress to fibrin coated ulcerations, fever, pharyngitis. Often accompanied by lesions on the hands and feet. Has seasonal patterna occuring MC in the summer/early fall periods. treatment is supportive as lesions self-resolve within 1 week. |  | 95 |
| 1487011304 | HSV gingivostomatitis | vesicles localized to the anterior oropharynx and lips. |  | 96 |
| 1141091419 | Hematuria (painless) | The most common complication of being a carrier for the sickle cell trait with 35-45% HbS. This complication is followed closely by isosthenuria. | 97 | |
| 1075454224 | Maternal estrogen effects | Mammary gland enlargement, swollen labia, non-purulent vaginal discharge (leukorrhea), and mild uterine withdrawal bleeding seen in a newborn are due to this effect. work-up is not necessary and reassurance should be provided. | 98 | |
| 300099048 | Brain CT scan (temporal brain abscess) | Test that should be performed with 4 y/o presenting with a significant PMH of recurrent otitis media and now with morning vomiting and nocturnal headaches. | 99 | |
| 1768304301 | Streptococcus pneumoniae (bacterial rhinosinusitis; > H. influenza > M. catarrhalis) | Most common cause of thick and persistent (> or equal 10 days) nasal discharge, nasal congestions, and cough. Yellow, purulent mucus dripping from posterior nasopharynx and nasal turbinates that are erythematous and edematous. | 100 | |
| 1092115525 | McCune-Albright syndrome (three P's) | Rare condition characterized by precocious puberty, cafe au lait spots and multiple bone defects (polyostotic fibrous dysplasia). Also may be associated with hyperthyroidism, prolactin or GH-secreting pituatary adenomas, and adrenal hypercortisolism. Caused by a sporadic mutation possibly in the G-protein cAMP-kinase of affected tissue resulting in autonomous activity of tissue. |  | 101 |
| 627572858 | Thyroid dysgenesis | MC cause of congenital hypothryoidism that presents with lethargy, hoarse cry, poor feeding, constipation, jaundice, dry skin, large fontanelles (majority asymptomatic). High TSH, low free T4. Most identified on screening. Tx levothyroxine. | 102 | |
| 2078342742 | microvesicular steatosis (reye syndrome) | 5 y/o had symptoms of low-grade fever, malaise, headache, cough, coryza, and sore throat (influenza B) 5 days prior and was treated with Aspirin. Now presents with lethargy, altered mental status, nausea vomiting. (-) jaundice or splenomegaly. Labs ALP 120; AST 358; ALT 410; ammonia 94 (nml <45); elevated PT and PTT. percutaneous liver biopsy shows this finding. | 103 | |
| 478947292 | Congenital syphilis (treponemia pallidum) | Newborn presenting with cutaneous lesions on palms and soles, hepatosplenomegaly, jaundice, anemia, and rhinorrhea. Radiographs demonstrate metaphyseal dystrophy and periostitis. >2y/o present with frontal bossing, high arched palate, hutchinson teeth, interstitial keratitis, saddle nose, perioral fissures. |  | 104 |
| 1756447017 | Renal biopsy | Test indicated with a child >10 y/o with nephrotic syndrome or minimal change disease that is unresponsive to steroids. | 105 | |
| 1284292290 | Pinealoma | 10 y/o presenting with limited upward gaze, ptosis, upper eyelid retraction, pupillary abnormalities. Obstructive hydrocephalus signs including papilledema, headache, vomiting, ataxia. Central precocious puberty. Serum and CSF show elevated a-fetoprotein and B-HCG. dx? | 106 | |
| 595028942 | Becker muscular dystrophy | Age of onse is typically 5-15 y/o with a mild muscle weakness. May later develop cardiomyopathy and death typically occurs by age 40-50 from heart failure. XR deletion resulting in decreased dystrophin. | 107 | |
| 1139551637 | Duchenne muscular dystrophy | Age of onset is typically 3-5 y/o with progressive weakness, Gower maneuver, calf pseudohypertrophy. High creatine phosphokines and aldolase. May also develop intellectual disability and cardiomyopathy. Usually require wheel-chair dependence by adolescence and death by 20-30 y/o from respiratory or heart failure. XR deletion resulting in absent dystrophin. | 108 | |
| 1702985848 | Normal | CSF analysis: WBC count (cells/uL) = 0-5; Glucose (mg/dL) = 40-70; Protein (mg/dL) = <40. What is the diagnosis? | 109 | |
| 966674319 | Bacterial meningitis | CSF analysis: WBC count (cells/uL) = >1000; Glucose (mg/dL) = <40; Protein (mg/dL) = >250. What is the diagnosis? | 110 | |
| 1546804782 | Tuberculosis meningitis | CSF analysis: WBC count (cells/uL) = 5-1000; Glucose (mg/dL) = <10; Protein (mg/dL) = >250. What is the most likely diagnosis? | 111 | |
| 1546804784 | Viral meningitis | CSF analysis: WBC count (cells/uL) = 100-1000; Glucose (mg/dL) = 40-70; Protein (mg/dL) = <100. What is the most likely diagnosis? | 112 | |
| 959522627 | Guillain-barre | CSF analysis: WBC count (cells/uL) = 0-5; Glucose (mg/dL) = 40-70; Protein (mg/dL) = 45-1000. What is the most likely diagnosis? | 113 | |
| 2152382672 | Orbital cellulitis | Pain with eye movements, proptosis, ophthalmoplegia, and diplopia. Bacterial sinusitis is the most common predisposing factor and may complicate to blindness, subperiosteal abscesses, cavernous sinus thrombosis, intracranial infection, and death. |  | 114 |
| 2152455953 | Strabismus | intermittant or constant ocular misalignment usually with esotropia (nasal deviation), but occasional esotropia (temporal deviation). First line treatment is to patch the normal eye (occlusion therapy) or blur vision of normal eye (penalization therapy) to prevent amblyopia. |  | 115 |
| 2152521605 | Trichotillomania | Recurrent hair pulling resulting in hair loss with repeated attempts to decrease/stop hair pulling and a history of significant distress or impairment. Treat is CBT. Often comorbid with OCD and family history of OCD. | 116 | |
| 2152572024 | Prostaglandin E1 | Conditions like severe coarctation of teh aorta, transposition of the great arteries, hypoplastic left heart syndrome, total anomalous pulmonary venous connection, and tricuspid atresia require this drug to maintain the PDA. | 117 | |
| 2155435168 | angiofibroma | Presents in adolescent with nasal obstruction, visible nasal mass, and frequent epistaxis. It is a benign growth capable of eroding and locally invading. | 118 | |
| 2163394107 | osteosarcoma | pain that worsens at night and is progressive. fever, weight loss, night sweats. erythema and enlargment over site. |  | 119 |
| 2163412317 | Ewing's sarcoma |  | 120 | |
| 2163426126 | ankylosing spondylitis | presents in late teens and early 20's with fatigue, intermittent hip pain, and LBP that worsens with inactivity and in the mornings. Anterior uveitis and heart block may occur. Tx indomethacin, exercise and improve posture, TNF inhibitors or sulfasalazine in refractory cases. | 121 | |
| 2164642424 | Phenobarbitol (complex partial seizures) | First line treatment in children for siezures that begin with auditory or visual hallucinations, deju vu, automatisms (lip smaking, chewing or evenn walking) then spreads bilaterally with aberrant electrical discharge causing impaired conciousness. Positive for postictal confusion/disorientation and amnesia are characteristic. | 122 | |
| 2164700557 | Infantile spasms | Male infant 6 months old brought to the hospital with muscular jerks and an uncle who had similar episodes when they were an infant. Abnormal interictal EEG characterized by hypsarrhythmia. medications like ACTH, prednisone, clonazepam, valproic acid may treat spasmas, but have little impact on long-term prognosis. Dx? | 123 | |
| 2164709019 | Lennox-Gastaut syndrome | Childhood onset seizures often refractory to treatment. presents with daily multiple seizures, often nocturnal tonic seizures appearing b/w age 2-6 y/o. More common in males and strong association with mental retardation, behavior disorders, delayed psychomotor development. Abnormal interictal EEG characterized by slow spike and wave complexes. | 124 | |
| 2164717616 | BPPV (benign paroxysmal positional vertigo) | Common cause of recurrent peripheral vertigo resulting in dislodged otolith that leads to disturbances in semicircular canals (95% horizontal, 5% horizontal). Presents with transient vertigo (lasting <1min) and mixed upbeat-torsional nystagmus triggered by changes in head position. N/V uncommon due to short lived. Dx by Dix-Hallpike maneuver. Tx Epley maneuver. subsides in wks to months, but 30% recur w/in a yr. antivertigo meds CI. | 125 | |
| 2164733486 | Labyrinthitis | Presents with unilateral tinnitus, ear fullness, or hearing loss along with acute onset of severe vertigo, head-motion intolerance and gait unsteadiness accompanied by N/V and nystagmus. Hx of viral illness 1 wk prior. PE shows abnormal vestibulo-ocular reflex, horizontal nystagmus always in 1 direction, no vertical misalignment by alternate covering. | 126 | |
| 2164742091 | vestibular neuritis | Acute onset of severe vertigo, head-motion intolerance and gait unsteadiness accompanied by N/V and nystagmus. Hx of viral illness 1 wk prior. PE shows abnormal vestibulo-ocular reflex, horizontal nystagmus always in 1 direction, no vertical misalignment by alternate covering. | 127 | |
| 2164785478 | Meniere's disease | REcurrent vertigo with auditory symptoms presenting more commonly in females. Episodes of severe vertigo, hearing loss, tinnitus, ear fullness often lasting hours or days. N/V typical. Progressive loss of low-frequency hearing over years and become deaf on affected side. Dx- 2 episodes > or equal to 20 minutes w/ remission of symptoms b/w episodes. Tx- migraine diets and meds. | 128 | |
| 2164798105 | Medulloblastoma | Presents as highly malignant (may seed in subarachnoid space) and may cause obstructive hydrocephalus. A primitive neuroectodermal tumor arising from fourth ventricle and causes increased ICP. Tx surgery + radiation + chemotherapy. | 129 | |
| 2164803135 | Ependymoma | Presents with low grade tumor possibly causing obstructive hydrocephalus. Arises from ependyma of ventricle (commonly the fourth) or the spinal cord. Tx surgery +/or radiation. | 130 | |
| 2164808539 | craniopharyngioma | Presents with benign tumor possibly causing hypopituitarism and is the MC suprasellar tumor in children. Imaging shows calcifications. Tx- surgery. | 131 | |
| 2164816491 | MRI with gadolinium | Imaging of brain, brainstem, and spine that should be done for presentation of cafe au lait spots, optic glioma, lisch nodules, kyphoscoliosis, and relatives with similar symptoms. Also conducting complete dermatoligic exam, ophthalmologic exam, and family hx. Clinically evident by 15-20 y/o. | 132 | |
| 2164827891 | Tuberous sclerosis | Infantile spasms and hypopigmented lesion on child's trunk, think this AD condition. | 133 | |
| 2164913678 | 17-hydroxyprogesterone (21-hydroxylase deficiency classic type) | Elevated serum concentration of this metabolite is diagnostic for a condition presenting with a female infant with hyponatremia, hyperkalemia, metabolic acidosis, ambiguous genitalia. possibly leading to life-threatening salt wasting. | 134 | |
| 2164925598 | MEN 1 | Pancreatic ilslet tumors (gastrinoma, insulinoma, VIPomas), parathyroid hyperplasia, pitutary adenomas. | 135 | |
| 2164928201 | MEN 2A | medullary carcinoma of thyroid, pheochromocytoma or adrenal hyperplasia, parathyroid gland hyperplasia. Due to AD RET proto-oncogene mutation. | 136 | |
| 2164931675 | MEN 2B | medullary carcinoma of thyroid, pheochromocytoma, oral and intestinal ganglioneuromatosis (mucosal neuromas), marfinoid habitus. Due to AD RET proto-oncogene mutation. | 137 | |
| 2164947489 | Campylobacter | MC bacterial diarrhea caused by ingestion of contaminated food or water affecting young children and young adults generally lasting 7-10 days. Fecal RBC's and WBC's frequently presenting with bloody diarrhea. Tx- erythromycin. | 138 | |
| 2164952111 | Clostridium difficile | Associated with recent ABx Tx (penicillins, quinolones, clindamycin) affecting primarily hospitilized pts. Presents with fever, abdominal pain, fecal RBC's, WBC's, sigmoidoscopy shows pseudomembranes. Complicates to toxic megacolon. Tx stop inciting ABx, begin metronidazole or vancomycin PO (or IV if not tolerated). | 139 | |
| 2164963535 | Entamoeba histolytica | Caused by ingestion of contaminated food or water. hx of travel to developing countries. incubation period can be 3 months. Presents with severe abdominal pain, fever, fecal RBC's, WBC's, endoscopy shows flask shaped ulcers. Chronic mimics IBD. steroids can lead to fatal perforation. Tx metronidazole. | 140 | |
| 2164972911 | E. coli 0157:H7 | Caused by ingestion of contaminated food (raw meat; unpasteurized juice). Affects children and elderly and generally lasts 5-10 days. presents with severe abdominal pain, low-grade fever, vomiting, fecal RBCs/WBCs. May develop renal failure and anemia. Avoid Abx or antidiarrheal as increase risk of complications. | 141 | |
| 2164980990 | Salmonella | Caused by ingestion of contaminated poultry or eggs affecting children and elderly generally lasting 2-5 days. presents with prodromal headache, fever, myalgia, abdominal pain, fecal WBCs. 5-10% of pts develop bacteremia. SCC risk of osteomyelitis. Tx PO quinolone or TMP-SMX | 142 | |
| 2164987765 | Shigella | Extremely contagious transmitted by fecal-oral route affecting young children and institutionalized pts. present with fecal RBCs/WBCs. may lead to severe dehydration, febrile seizures. Tx TMP-SMX. | 143 | |
| 2164995275 | hernia | Leading cause of small bowel obstruction in children. | 144 | |
| 2165081738 | constitutional growth delay | Presents with primary amenorrhea, decreased GnRH, decreased LH/FSH, decreased estrogen/progesterone (prepuberty levels). | 145 | |
| 2165085674 | Hypogonadotropic hypoganadism (pituitary failure) | Presents with primary amenorrhea, decreased GnRH, decreased LH/FSH, decreased estrogen/progesterone | 146 | |
| 2165090479 | Hypergonadotropic hypogonadism (ovarian failure) | Presents with primary amenorrhea, increased GnRH, increased LH/FSH, decreased estrogen/progesterone. | 147 | |
| 2165095116 | Anovulatory problem (PCOS or estrogen receptor) | presents with primary amenorrhea, increased GnRH, increased LH/FSH, incresaed Estrogen/progesterone. | 148 | |
| 2165097092 | Anatomic problem (mullerian agenesis, imperforate hymen) | Presents with primary amenorrhea and normal GnRH, LH/FSH, estrogen/progesterone. | 149 | |
| 2171236374 | Drug allergic reaction | Erythematous maculopapular rash generally confluent, hives less frequent; onset from 1-2 days to 1 wk after starting medication; pruritic, afebrile, speads distally. Tx- antihistamines or GC's treats over 1-2 wks. complicates to interstitial nephritis or TEN. |  | 150 |
| 2171243960 | Urticaria | General hives, wheals, via acute dermal edema and erythema(IgE mediated, histamine release), pruritic, erythematous borders with pale centers usually lasts <24 hrs. |  | 151 |
| 2171264766 | Varicella | Common and usually benign disease, >90% of children infected by age 10, highly infectious via vesicles or respiratory tract (coughing, sneezing). 1-3 wk incubation; Prodrome- fever, headache, flu like symptoms, malaise; Rash- red macules, vesicles (1-2 mm) w/ light red erythematous base which may follow flu like symptoms after 1-2 days vesicles become pustular and form crusts/scabs. start on trunk spread to excremeties. Labs- low WBC. CI- ASPIRIN. |  | 152 |
| 2171282105 | Measles (rubeola) | Incubation 1-2 wks; prodrome 3-4 days w/ cough, coryza, conjunctivitis, fever, weakness, congestion, photophobia, rhinitis; rash- enlargin red/pink fine spots starting over forehead, behind ears, and upper neck, spreading towards face, trunk, and then arms (koplik spots); rash associated with fever, rash disappears with fine skin peeling. complicates- conjunctivits, otitis media, hearing loss, diarrhea, pneumonia, encephalitis, seizures. Tx- Vit A. |  | 153 |
| 4185012197 | Biliary atresia (direct hyperbilirubinemia develops with jaundice in first 2 months of life; MC indication for liver transplant in pediatrics; conjugated hyperbilirubinemia always pathologic; first get an U/S) | Most likely cause of a 3wk old infant jaundice for last 1 week. + acholic stools/dark urine, hepatomegaly, conjugated hyperbilirubinemia, mild elevation in transaminases. | 154 | |
| 4185052963 | Breast milk jaundice (generally benign, but may require phototherapy) | Most likely cause of jaundice in a 3 wk old infant with jaundice for last 1 week. + Unconjugated hyperbilirubinemia (10-30mg/dL). | 155 | |
| 4185072744 | Physiologic jaundice (resolves after 1 week) | Most likely cause of jaundice appearing in the first 24 hrs of life in a neonate. + unconjugated hyperbilirubinemia. | 156 | |
| 4185102753 | Alloimmune hemolytic anemia (erythroblatosis fetalis; maternal ABO or Rh or minor blood group antigens) | Most likely cause of jaundice in a neonate with + unconjugated hyperbilirubinemia, positive coombs test, anemia. | 157 | |
| 4185148778 | ventricular septal defect | Most likely cause of a heart murmur in a neonate presenting with micrognathia, microcephaly, rocker bottom feet, overlapping fingers and absent palmer creases. | 158 | |
| 4185162343 | congenital heart block (neonatal lupus) | Heart condition most likely with a mother who presented with malar rash and hematuria during pregnancy. She was found to be + anti-dsDNA. | 159 | |
| 4292540952 | Ebstein's anomaly (atrialization of right ventricle and malformed tricuspid valve) | Lithium exposure in the first trimester of pregnancy is associated with an increased risk development of this condition in the fetus. | 160 | |
| 4292655136 | Hepatitis B immune globulin and hepatitis b vaccine | These two steps should be taken following the confirmed vertical transmission of hepatitis B from a pregnant mother to the neonate. | 161 | |
| 4594142619 | chemical irritation (conjunctivitis) | Most likely cause of conjunctivitis in a neonate after 1 day post delivery. | 162 | |
| 4594144039 | Neisseria gonorrheae (conjunctivitis; tx ceftriaxone) | Most likely cause of conjunctivitis in a neonate after 2-7 days post delivery. | 163 | |
| 4594146499 | Chlamydia trachomatis (conjunctivitis; tx oral erythromycin) | Most likely cause of conjunctivitis in a neonate after more than 7 days post delivery. | 164 | |
| 4594148796 | herpes (conjunctivitis; systemic acyclovir and topical vidarabine) | Most likely cause of conjunctivitis in a neonate after 3 weeks or more post delivery. | 165 | |
| 4594411282 | glucose-6-phosphatase deficiency (von gierks) | Most likely diagnosis of a 3-4 month old infant presenting with hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia on labs. physical exam shows doll-like face, thin extremeties, short/protuberant abdomen with enlarged liver and kidneys. | 166 | |
| 4594421602 | acid maltase deficiency (pompe disease) | Most likely diagnosis of a 3 week old presenting as a floppy-baby since birth with feeding difficulties, macroglossia, heart failure, and hepatomegaly. | 167 | |
| 4594429590 | glycogen debranching enzyme deficiency | Most likely diagnosis of a newborn presenting with hepatomegaly, hypoglycemia, hyperlipidemia, growth retardation, increased liver transaminases, fasting ketosis, normal blood lactate and uric acid. | 168 | |
| 4594442718 | glycogen branching enzyme deficiency | Most likely diagnosis of an 18 month old with hepatosplenomegaly and failure to thrive. Progresses to cirrhosis of liver. | 169 | |
| 4594593573 | HBIG (hepatitis B immunoglobulin) | additional treatment option for an infant with a mother who was positive HBs-Ag in addition to a vaccine. | 170 | |
| 4594717366 | 4 hours | If tachypnea lasts longer than this period of time in a newborn it is considered sepsis and must be followed up with a blood and urine culture. A lumbar puncture is done only if signs of irritability, lethargy, temperature irregularity, and feeding problems. | 171 | |
| 4599066510 | Mccune albright syndrome | Most likely diagnosis with a clinical presentation of precocious puberty, cafe au lait spots, and multiple bone defects (polyostic fibrous dysplasia). Accounts for 5% of female precocious puberty and may be associated with hyperthyroidism. | 172 | |
| 4599140742 | friedreich ataxia (trinucleotide repeat AR; tocopherol transport protein) | Most likely diagnosis with a clinical presentation of a child with progressive limb and gait ataxia, dysarthria, loss of joint position and vibration senses, absent tendon reflexes in the legs, and extensor plantar responses. Typically develop myocarditis/CHF, are wheelchair bound by 25yo, and dead by 30-35 yo. | 173 | |
| 4599328224 | wilms tumor (WAGR- wilms tumor, aniridia, genitourinary malformations, mental retardation) | Most likely etiology of an abdominal mass felt in an infant with a history of vomiting, constipation, hypospadias, delayed mental development and the pictured symptoms. |  | 174 |
