2018 Pearls Flashcards
1
Q
Contraindications to rotavirus vaccine administration are…
A
Infants with a personal history of intussusception or a personal history of an anaphylactic reaction to the rotavirus vaccine should not receive the vaccine.
2
Q
There are age limits beyond which the Rota vaccine should not be administered
A
(14 weeks, 6 days for a first dose, 8 months, 0 days for the second or third doses); thus, delaying the vaccine could result in missed opportunities for protection
3
Q
It is critical to order______ blood products for all immunocompromised individuals, such as newborns and patients with cancer who are receiving chemotherapy to limit the risk of transfusion associated GVHD .
A
irradiated
Sx: skin (rash) elevated LFT/bili, diarrhea
4
Q
Irradiation of blood products
A
damages the DNA of donor lymphocytes, thereby rendering them replication incompetent and definitively preventing transfusion-associated graft-vs-host disease.
5
Q
____ are stereotyped motor movements usually involving the face and upper extremities. They are accompanied by an internal compulsion to do the movement and a sense of relief afterwards.
A
Motor tics
Simple motor tics are brief, individual movements such as shoulder twitching, forceful eye blinking, or eye rolling.
6
Q
The presence of hepatitis B surface antigen and total antibody to hepatitis B core antigen without IgM hepatitis B core antibody is indicative of
A
chronic hepatitis B virus infection.
7
Q
The presence of _____ suggests high viral replication and increased risk of hepatitis B virus transmission.
A
hepatitis B e antigen
8
Q
Facial angiofibromas in patients who have tuberous sclerosis complex may mimic the lesions of acne vulgaris, can become large and disfiguring, and often respond to treatment with
A
topical sirolimus.
9
Q
In primary adrenal insufficiency, there is a deficiency of both glucocorticoid (cortisol) and mineralocorticoid (aldosterone). In response to low cortisol levels, _____ levels are high.
A
adrenocorticotropic hormone (ACTH) this causes hyperpigmented skin
10
Q
Fatigue, nausea, weight loss, hypotension, volume depletion, and diffuse hyperpigmentation are common presenting symptoms and signs of ____
What lab findings do you expect?
A
Addison disease hypONa hypERK hypOglycemia metabolic acidosis low cortisol high ACTH eosinophelia
11
Q
First line medication for depression in teens
A
fluoxetine
you can do escitolopram if this does not work well, taper and then switch to the other SSRI, trial for 4 weeks
Stay on minimum of 6 months
12
Q
4 month old with hepatomegaly, renomegaly, and recurrent episodes of hypoglycemia with a doll-like facies with chubby cheeks, thin extremities, distended abdomen, and short stature.
Dx?
Inheritance pattern?
A
Glycogen storage disease type 1
Autosomal recessive
13
Q
In glycogen storage disease type 1 what labs abnormalities do you see?
A
hypoglycemia, lactic acidosis, hyperlipidemia, hypertriglyceridemia, and hyperuricemia.
14
Q
autosomal recessive lysosomal storage disorder that has a spectrum of presentations but universally includes bone disease, hepatosplenomegaly, cytopenias, and pulmonary disease.
A
Gaucher disease
15
Q
autosomal recessive lysosomal storage disorder
- coarsening of facial features over time but normal appearance at birth
- umbilical and inguinal hernias
- frequent upper respiratory infections, skeletal involvement (dysostosis multiplex), hearing loss, hepatosplenomegaly, valvular cardiac disease, and progressive intellectual disability.
A
Hurler syndrome
16
Q
Infants with these disorders exhibit hypotonia, distinctive facies, poor feeding, seizures, and hepatic dysfunction. As the disease progresses, retinal dystrophy, sensorineural hearing loss, and progressive developmental disability occur
A
Peroxisomal biogenesis disorders
17
Q
Autosomal recessive disorder that presents with severe hypotonia, muscular weakness, cardiomegaly with progression to cardiac failure, failure to thrive, and respiratory distress.
What disease is this?
What tests do you get?
A
Pompe
–elevated creatine kinase level and urinary oligosaccharides
18
Q
Children with _______ have recurrent episodes of hypoglycemia, lactic acidosis, hyperlipidemia, hypertriglyceridemia, and hyperuricemia.
A
glycogen storage disease
19
Q
Pediatric hypertension is defined as the sustained elevation of systolic or diastolic blood pressure
A
between the 90th and the 95th percentile for a child’s age, sex, and height, or between 120/<80 mm Hg and 129/<80 mm Hg
20
Q
Children who are symptomatic or who have secondary hypertension, ventricular hypertrophy, hypertensive retinopathy, or diabetes mellitus should be
A
started on antihypertensive medications in addition to lifestyle modifications.
21
Q
When do we start ABx on burn patients?
A
Antibiotics for burn injury patients should be reserved for clinical situations with clear evidence of an active infection. A change in the appearance of the wound, including new discoloration, is the most suggestive of an infection.
22
Q
What are some expected changes in vitals in burn patients?
A
Extensive burns produce a systemic inflammatory response that results in intravascular hypovolemia from capillary leak and a hypermetabolic state. As a result, vital sign abnormalities, including fever, tachycardia, and tachypnea, are common in patients with burn injuries.
23
Q
Kiddo who is a mouth breather, mucoid nasal discharge and on exam has grape like lesions in BL nares
ML diagnosis
A
Nasal polyps
CF… this is the MCC of nasal polyposis in children
24
Q
______ infections typically occur during the summer and early fall, and infants and young children are at highest risk. Transmission occurs via fecal-oral contact. can cause HFM, respiratory illness, aseptic meningitis, GI symptoms or much more severe in neonates or immunocompromized
A
Enterovirus
25
Pt with diarrheal illness 7 days ago, seems to be doing better but still having 4-7 episodes diarrhea/day, worse right after meals and abdomen is distended . What is likely causing this?
Secondary lactose intolerance; can be supported by low stool pH and elevated stool reducing substances
26
12yo with pain in right knee with running, jumping, and kneeling. On physical examination,tender and prominent tibial tuberosity. Pain with extension of the knee against resistance.
Osgood-Schlatter
disease is an apophysitis of the tibial tuberosity
Tx stretching, modified activity and patellar strap
27
Most common organisms implicated in infective endocarditis?
Staph Aureus more common in kid with 'normal heart'
| Strep viridans more associated with abnormal cardiac valves (CHDx, Rheumatic heart dx, post op)
28
Signs and symptoms of_____ deficiency may include hyporeflexia, ataxia, decreased vibratory sensation and proprioception, muscle weakness, and limitation of eye movement
vitamin E
29
Congenital neutropenia PLUS
| short limbs, dwarfishm, fine hair, shitty immune system
Cartilage-hair hypoplasia
30
Congenital neutropenia PLUS varying degrees of oculocutaneous albinims
Chediak Higashi
31
Congenital neutropenia PLUS FTT, exocrine pancreatic dysfunction (diarrhea) and skeletal anomalies
Shwachman-Diamond syndrome
32
All international adoptees and refugees coming to the United States with unexplained eosinophilia must undergo serologic testing for ______after exclusion of common pathogens associated with eosinophilia.
Strongyloides stercoralis
33
Newborn with moderate tachypnea but lack of respiratory distress and >10 point differential between her pre- and postductal saturations suggest a cardiac cause of hypoxemia. CXR shows decreased pulmonary markings and LVH on EKG
Newborn with tricuspid atresia
34
14yoM worried about height. Growth curve shows consistently at about 3% for height and weight, bone age is 11 years old, still Tanner 1.
CONSTIUTIONAL growth delay (see delayed bone age that matches height and delayed puberty)
expect to reach normal adult height, no treatment
MIGHT consider testosterone
35
Who do we give growth hormone to?
Children with idopathic short stature with height > 2.25 SD below mean who are unlikely to catch up.
36
Children between the ages of about 1 and 5 years are especially susceptible to “toddler fractures,” which are
oblique fractures of the distal tibia.
37
A loud systolic murmur at the right upper sternal border and associated with a click best fits a diagnosis of
aortic stenosis
38
systolic murmur caused by increased flow across the pulmonary valve; this murmur occurs during ejection at the upper left sternal border. A fixed split S2 is typical
ASD
39
abrupt onset of pharyngitis, palpebral conjunctivitis, fever, moderate degree of illness, and preauricular lymphadenopathy.
Adenovirus
40
pharyngitis with vesicles or ulcers; exudate is less commonly seen. A variety of exanthems and enanthems are associated
Coxsackievirus and echovirus
41
Hemolytic uremic syndrome is characterized by the triad of
```
microangiopathic hemolytic anemia, thrombocytopenia, and kidney injury.
See schistocytes/burr cells on smear
Uremia on renal function
Coombs negative with normal complement
Caused by shiga toxin producing E.coli
```
42
_______ should be considered when skin lesions in the diaper area and scalp persist despite frontline therapy
Langerhans cell histiocytos
in some children, multifocal can involve the pituitary stalk and lead to diabetes insipidus with associated increased thirst and urinations
43
Pt in southwest presenting with malaise, cough, myalgias, fever, headache, and chest discomfort. Can see cutaneous manifestations or pulmonary findings like pleural effusion
Dx
How to Dx
Coccidiomyocosis
| Immunodiffusion and complement fixation studies from serum are highly specific for coccidioidomycosis.
44
leading bacterial cause of foodborne gastroenteritis in children.
Present with bloody diarrhea, vomitting and dehydration. History of consumption of unpasteurized cow milk
Campylobacter
Tranimission via foodborne (unpasturized milk, water, undercooked poultry)
Can tx with azithro or erythro to limit duration
45
Child presents with tachycardia, tachypnea, hypotension, lethargy, and toxic appearance combined with a characteristic purpuric rash.
Likely dx?
Tx of choice?
Neisseria meningitidis
gram-negative encapsulated diplococcus
Tx is 3rd gen cephalosporin~ ceftriaxone
46
In children and adolescents,_____ most often presents with erythematous papules and plaques covered by a thick, adherent scale. The extensor surfaces of the elbows and knees are commonly involved
psoriasis
tx with topical corticosteroids
Psoriasis is a papulosquamous (ie, elevated lesions with scale) disorder likely caused by a genetic predisposition and an environmental trigger (like infection or trauma). It is believed to be an immune-mediated inflammatory process characterized by epidermal hyperplasia.
47
Patient with HYPOnatremia, HYPO-osmolality, euvolemia or mild hypervolemia, inappropriately concentrated urine, and urine sodium excretion
SIADH
See euvolemia or mild hypervolemia (cerebral salt wasting has HYPOvolemia)
Tx fluid restrict
48
Causes of SIADH
SIADH can be associated with central nervous system and pulmonary disorders; hypothyroidism; glucocorticoid deficiency; and medications, including carbamazepine, selective serotonin reuptake inhibitors, tricyclic antidepressants, vincristine, and cyclophosphamide
49
In order to qualify for IEP you must
In order to qualify for 504 you must
Is grade retention beneficial
IEP: must have LD identified
504: must have medical diagnosis like ADHD, ect.
grade retention not helpful, more tutoring and after school programs
50
Who benefits from preventative youth violence elimination programs?
Universal prevention approaches that have a positive impact on all youth are more likely to be effective in eliminating youth violence than programs targeting only at-risk youth.
Mentoring programs have been shown to decrease adolescent risk behaviors to include violence and delinquency.
51
What are LLSB murmurs?
VSD:holosystolic
Stills: mid-systolic, buzzing/musical and non-radiating, benign and at apex that diminishes w/ standing
HOCM: laterally displaced PMI, mid-systolic and increases w/ valsalva
Tricuspid Stenosis: Mid-diastolic (associated with Rheumatic fever)
52
Only ___% of infants born to mothers with Hep C will aquire the infection.
Those that do are at risk for...
5%
Increased risk for chronic infection and liver fibrosis during childhood
slight increase risk for HCC
Monitor with LFTs and if significant disease burden should get annual HCC screening with US and alfa-feto-protein
53
Among children with persistent otorrhea, 25% to 60% are found to have a
cholesteatoma, a collection of squamous epithelial cells and keratin within the middle ear.
54
Congenital heart disease lesions with left-to-right shunting will manifest at appx _____
2 months
-VSD, AV canal defects, anomalous pulmonary venous return
As the pulmonary vascular resistance decreases, the amount of shunted blood increases. Because the left ventricle pumps blood through the shunt, as well as the forward systemic flow, congestive heart failure ensues.
55
The treatment for a neonate with ductal-dependent circulation is to start a ________ which opens the ductus arteriosus. This therapy should be started without delay if a ductal-dependent lesion is suspected, even before echocardiography results are available.
prostaglandin infusion
56
Overt masturbation increases through early childhood, peaking at age____
Children cease masturbating in public when
5 years
they become aware of social norms
*Redirection and behavior modification may be used to help limit masturbation behaviors to private settings.
57
Marfan syndrome is a connective tissue disorder that typically leads to musculoskeletal, cardiovascular, skin, and eye abnormalities. This condition is caused by a mutation in the ____ and is inherited in an _____. In about one-quarter of affected individuals, Marfan syndrome occurs because of a spontaneous mutation
fibrillin-1 (FBN1) gene
| autosomal dominant fashion
58
Athletes with Marfan syndrome are at risk for ______
What do we screen them with?
Can they play sports?
aortic root rupture and aortic dissection
Echo
Individuals with a diagnosis of Marfan syndrome without aortic dilation or significant valvular disease may be cleared to participate in sports but should avoid high-intensity sports, especially those with intense isometric activity such as weight-lifting.
59
group of genetic conditions characterized by Pierre Robin sequence (cleft palate, glossoptosis, and micrognathia or retrognathia) and severe myopia or other ocular abnormalities, as well as potential sensorineural hearing loss and skeletal abnormalities.
Stickler syndrome
60
Children with orofacial clefts are at increased risk of ________; this outcome tends to be improved with earlier surgical correction.
language and articulation disorders
61
Lip clefts are typically repaired before_____, with palatal cleft repair occurring around ____.
6 months of age
| 1 year of age
62
The therapies for children with Graves disease include?
antithyroid drugs (methimazole as the first-line treatment), radioactive iodine therapy, and thyroidectomy
β-Blockers such as propranolol are indicated in patients with a heart rate greater than 100 beats/min, palpitations, hypertension, or tremors
Get LFT and CBC prior to starting therapy
63
Recommended as first-line tests by the American College of Medical Genetics and the American Academy of Pediatrics for children with global developmental delay and intellectual disability of unknown etiology.
Chromosomal microarray and fragile X testing
64
Isolated prolonged PTT with bleeding/bruising tendencies should prompt workup for...
Von Willebrand
| MC heritable bleeding disorder; links fibrin to functional platelets to make clot
65
Pt exposed to measles. She is 5 months old.. next step?
| What if she was 9 months?
If <6 mo give measles IG (w/i 6 days exposure)
| If >6mo but <12 mo give MMR and will still need normal schedule (w/i 72 hrs of exposure)
66
Screening tests for diabetes include a
fasting plasma glucose (≥125 mg/dL [6.9 mmol/L] is diagnostic), 2-hour plasma glucose during a 75-g oral glucose tolerance test (≥200 mg/dL [11.1 mmol/L] is diagnostic), or hemoglobin A1c (≥6.5% is diagnostic)
67
When does a patient get tetanus IG?
1. For DIRTY wounds AND IF <3 or Unknown number of tetanus vaccines
68
Diabetes insipidus occurs secondary to decreased secretion of ______ or secondary to renal resistance to ________
```
antidiuretic hormone (central DI)
antidiuretic hormone effects (nephrogenic DI).
```
69
Patients with _____exhibit polyuria, polydipsia, and increased thirst. They may exhibit varying degrees of dehydration, and laboratory evaluation usually reveals hypernatremia in association with dilute urine (urine osmolality < plasma osmolality).
DI
70
______in children is secondary to a mutation in either the antidiuretic hormone receptor, arginine vasopressin receptor 2, or the aquaporin 2 channel.
Nephrogenic DI
71
In Nephrogenic DI, the arginine vasopressin receptor 2 mutations have an ____inheritance and account for 90% of cases
X-linked *
| male individuals are more severely affected than female individuals.
72
Children with CKD usually have poor growth as a manifestation of decreased renal function. Reduced renal erythropoietin production with CKD leads to anemia. The anemia is _____and ______ consistent with anemia of chronic disease; however, these patient are at increased risk for_____ and _____anemia because of the poor nutritional status associated with advanced stages of CKD.
normocytic and normochromic
iron- and vitamin B12–deficient
73
What are two screening tests for syphillis?
| If positive what is the next step?
Nontreponemal test: RPR or VDRL (good for screening and monitoring)
Must confirm with treponemal test: FTA-ABS (fluorescent treponemal AnitB testing)
DEFINITIVE is darkfield but not widely available
74
β-Blockers have side effects
of bradycardia, hypotension, hypoglycemia, hypoglycemia-induced seizures, bronchospasm, and hyperkalemia
75
Most ______ infants will have attained the following milestones: sits without support; displays stranger anxiety; engages in back-and-forth play; pulls to stand and cruises along furniture; demonstrates a 3-finger grasp; and speaks with polysyllabic babbling plus nonspecific “mama.”
9-month-old
76
The innate immune system is comprised of nonspecific barrier defense (skin, hair, mucosal barrier) and cellular defense (neutrophils, macrophages, natural killer cells). The adaptive immune system is comprised of .....
specific humoral immunity (B cells) and cellular immunity (T cells).
77
Agammaglobulinemia is an ___defect of the adaptive humoral immune system that presents with
X-linked genetic
| invasive bacterial infections in the first year after birth
78
Patients with agammaglobulinemia require treatment with
replacement immunoglobulin subcutaneously or intravenously every 3 to 4 weeks for life, unless they undergo a hematopoietic stem cell transplant.
79
Why is earlier implantation of cochlear implants preferred?
Implantation of cochlear implants by 12 months of age achieves optimal language and educational outcomes; therefore, early identification and referral for evaluation and treatment are particularly important.
80
In _______, the typical seizure is focal with rhythmic twitching of one side of the face and the ipsilateral arm, with prominent drooling. The child often cannot speak or has repetitive chewing or swallowing during the seizure.
When do they occur?
Tx?
Benign rolandic epilepsy (BRE)
IG during sleep
See periolandic (centrotemporal) spikes
often resolve by 16-18 yo, usually don't need treatment
81
Neonates requiring positive pressure ventilation should have ....
The initial pressure required to inflate the lungs after birth may be as high as ...
oxygen saturation monitored concurrently with pulse oximetry on the right hand.
40 mm Hg.
Try not to go jack up FiO2, but def go to 100% if you are intubating
82
Psychoeducational tests, such as IQ tests and achievement tests, generally have a mean score of 100 and standard deviation (SD) of 15. The average range is
within 1 SD of the mean (85–115)
IQ test scores greater than 2 SD below the mean (<70) in both cognitive and adaptive measures are in the intellectually disabled range
83
defined as a meaningful discrepancy between intelligence scores and achievement scores, low achievement in the setting of at least low average intelligence, or a student’s failure to respond to evidence-based educational interventions.
Learning Disability
84
5 year old presents with hepatosplenomegaly, anemia, thrombocytopenia, bone pain, pathologic fractures, radiologic bone evidence of disease, and lung disease. Normal appearance and neuro exam.
Diagnosis?
Further studies?
Gaucher disease type 1
Autosomal recessive
Get gjucocerebrosidase enZ activity and genetics study
Tx enZ replacement and partial/total splenectomy
85
that presents with coarsening of facial features, progressive intellectual disability, progressive airway disease, cardiac disease, short stature, conductive and sensorineural hearing loss, and dysostosis multiplex on skeletal survey.
Dx?
Genetics?
Hunter syndrome
X-linked lysosomal storage disorder
Confirm dx with abnormal urine glycosaminoglycans and skeletal survey + deficiency iduronate 2 sulfate activity
86
Passive immunoprophylaxis with varicella-zoster immune globulin is indicated for who?
Passive immunization should be administered as soon as possible within ____days after exposure.
VZV-exposed patients who lack evidence of immunity to VZV and who are immunocompromised or not candidates to receive the live virus varicella vaccine.
10 days
Population: primary/acquired immunodeficiency, on immunosuppression, pregnant, newborns in fmom with VZV 5 days before/2 days after deliver, premature infants >28wk with non immune moms
87
When should a kiddo be kept from school in regards to diarrhea, when can they go back?
Medical evaluation for stools with blood or mucus; exclusion until stools are contained
in the diaper or when toilet-trained children no longer have accidents using the toilet and when stool frequency becomes less than 2 stools above that child’s
normal frequency/24 hours.
88
How do loop diuretics work?
| Thiazides?
Loop diuretics (furosemide, bumetanide) inhibit the Na+-K+-2Cl- channel in the thick ascending loop of Henle. Thiazide diuretics inhibit the Na+-Cl- cotransporter in the distal tubule and connecting segment.
89
What are the electrolyte disturbances that occur 2/2 to loop diuretics?
Volume contraction, hypokalemia, metabolic alkalosis, and hyperuricemia commonly occur with loop and thiazide diuretic therapy.
90
The differential diagnosis for conjugated hyperbilirubinemia includes
urinary tract infection; genetic syndromes such as Alagille syndrome, galactosemia, total parenteral nutrition–associated cholestasis, ɑ1-antitrypsin deficiency, medication side effect; and congenital anomalies such as biliary atresia
91
Toe-walking is a normal variant in children younger than ____ years and no treatment is warranted in this age range.
3
Unilateral toe-walking should always be evaluated further, because it may indicate the presence of hemiplegia, developmental dysplasia of the hip, or leg-length discrepancy.
92
In the acute phase of respiratory acidosis...
| In the chronic phase of respiratory acidosis, over days or longer...
Acute: serum bicarbonate rises slightly (approximately 1 mmol/L for every 10 mm Hg of increased partial pressure of carbon dioxide [Pco2]) due to buffering by intracellular proteins.
Chronic: serum bicarbonate rises approximately 3.5 mmol/L for every 10 mm Hg of increased Pco2, because of the increased reabsorption of bicarbonate at the renal tubular level.
93
Precocious puberty (ie, before the age of 9 years) occurs rarely in boys but merits evaluation because it may be due to a pathologic cause and may
compromise final adult height
94
Recommendations for typanostomy tube
- Bilateral OME lasting at least 3 months together with conductive hearing loss
- Bilateral or unilateral OME lasting at least 3 months together with risk factors for speech, language, or learning problems (eg, neurodevelopmental disabilities, craniofacial anomalies)
- Bilateral or unilateral OME lasting at least 3 months together with symptoms or conditions such as pain, vestibular problems, tympanic membrane damage, or middle ear damage
- Recurrent AOM with OME at the time of assessment
95
Indications for surgical tympanostomy tube removal include:
- Failure of the tube to extrude more than 3 years
- Migration of the tube into the middle ear
- Tube-associated granulation tissue that does not respond to treatment
- Chronic otorrhea that does not respond to treatment
- Resolution of the condition that prompted insertion (eg, repair of cleft palate), especially in an older child
96
______increases the chance of thromboembolic events in patients with cyanotic congenital heart disease
Polycythemia
97
```
Glomerulonephritis with
LOW C3
NORMAL C4
Dx?
Biopsy?
Tx?
```
post infectious glomerulnephritis
follow C3 levels; if don't normalize consider alternative dx
Bx (which is not necessary) lumpy bumpy IgG deposits
Tx: steroids or cyclophosphamide
98
```
Glomerulonephritis with
LOW C3
LOW C4
Dx?
Biopsy?
```
Membranoproliferative glomerulonephritis
| Tram Track on Bx
99
```
Glomerulonephritis with
LOW C3
LOW C4
+ANA
Dx?
```
Systemic lupus nephritis
| Tx steroids and cyclophosphamide`
100
Avulsed permanent teeth should be _______. Prognosis is best when reimplantation is completed within ____
immediately reimplanted
15 to 30 minutes of avulsion.
Avulsed PRIMARY teeth are generally not reimplanted, as this may actually damage the developing permanent tooth.
101
In children with bacterial meningitis, a decreased level of consciousness at presentation increases the risk for
long-term neurologic sequelae or death...
| developmental delay, intellectual disability, hearing impairment, epilepsy, spasticity, and hemiparesis
102
Neonatal alloimmune thrombocytopenia results from
maternal antibody production to an antigen expressed on fetal platelets that is not expressed on maternal platelets. If severe, it is treated with a transfusion of maternal platelets because they do not express the responsible antigen.
Subsequent children born to parents of a neonate with neonatal alloimmune thrombocytopenia are at risk to develop this condition
103
In premature neonates, fluctuations in systemic blood pressure can cause acute increases and decreases in cerebral perfusion that increase the risk of
bleeding into the vascularized germinal matrix, resulting in IVH
REDUCED with antenatal steroid administration and delayed cord clamping
104
Presents as an acquired patch of alopecia that contains black-dot hairs or scale.
Dx?
Tx?
Tinea capitis
| Tx: griseofulvin, terbinafine or fluconazole
105
Intimate partner violence occurs across all demographic groups, and risk factors include
young age, lower scioeconomic status, female gender, and personal or family history of violence. Teens and pregnant women are at particularly high risk for intimate partner violence.
106
Pt with elevated direct bili and LFTs, declining school performance and bilateral UE tremor.
Concerning for?
How to diagnose?
Tx?
Wilson's disease
Dx with liver biopsy but supported by low serum copper and low ceroplasmin (screen family members)
Tx Penicillimine
107
Gilbert results in elevated _____ bilirubin
INDIRECT
108
Biochemically, hypoparathyroidism manifests as
Ca?
PTH?
Phos?
hypocalcemia, low parathyroid hormone (PTH), and high phosphorous levels. Phosphorous levels are high because, in addition to maintaining normal serum calcium levels, PTH also causes phosphate excretion in the kidney.
109
```
Very long-chain acyl-CoA dehydrogenase deficiency is a fatty acid oxidation disorder with a severe early onset presentation of .....
Laboratory abnormalities include
Glucose?
Acid base?
CK?
ammonia?
```
hypoketotic hypoglycemia, cardiomyopathy, hepatomegaly, and hypotonia.
Hypoglycemia, metabolic acidosis and mild to moderate elevation of creatine kinase, liver function test results, and ammonia levels as well as C16:1, C14:2, C14:1, and C18:1 acylcarnitine
Tx: high-dose intravenous glucose, hydration
110
typically presents with coarse facies, developmental regression, skeletal dysostosis multiplex, frequent upper respiratory infections, umbilical/inguinal hernia, hepatosplenomegaly, and short stature in the absence of hypoglycemia, liver failure, or metabolic acidosis.
Lysosomal storage disorder
111
The minimum diagnostic criteria for pelvic inflammatory disease (PID) are
pelvic examination findings of cervical motion tenderness/ uterine tenderness/ adnexal tenderness. In young women presenting with abdominal or pelvic pain, any 1 of these findings is sufficient to prompt treatment for PID provided other diagnoses have been excluded.
112
Tx for PID outpatient
| Tx for PID inpatient
Outpatient: Ceftriaxone 250mg IM x11 dose PLUS Doxy 100mg PO BID x14 days +/- Metronidazole for 14 days (anaerobe coverage)
Inpatient: Cefoxitin + Doxy BID x14 OR
Clinda + Gent OR
Unasyn + Doxy
113
follow a population of patients with a disease over time. This approach is likely to yield important information about the natural history of a disease
Cohort studies
114
Useful to study individuals with a disease compared to individuals without the disease to evaluate risk factors and outcomes for the disease.
Case-control study
115
Two most common causes of non-anion gap metabolic acidosis
Chronic diarrhea vs RTA
~ due to loss of HOC3- (bicarb) either in stool or kidney
ALL RTAs have metabolic acidosis and decreased renal ammonium (NH4 excretion)...a urinary anion gap can act as surrogate marker for urinary ammonium excretion
116
Pt from S.America with intermittent seizures. Has brain imaging showing a ring enhancing lesion
Dx?
How to dx?
Tx?
Neurocysticercosis from Taenia solium
Seen in latin america or asia
Dx with serology or serum antiBody for laral Taenia solium
117
AFter first simple febrile seizure in infant >12 mo the risk of recurrence is ___
Risk of epilepsy is ____
30%
| risk of epilepsy is ~2%
118
Diagnostic criteria for anorexia nervosa (AN) include:
1. Restriction of energy intake resulting in significantly low body weight
2. Intense fear of gaining weight
3. Severe body image distortion with denial of the seriousness of the illness
119
Drugs that cause hypertension include
Steroids, decongestants, NSAIDS, β-adrenergic agonists, EPO, cyclosporine, tacrolimus, and stimulants
120
The recurrence risk for neural tube defect in a future pregnancy with 1 sibling affected and neither parent affected is closest to ____.
If 2 siblings were affected, the recurrence risk would approach ___ to ____.
5%
| 10% to 12%
121
Increased PT alone with bleed
Factor VII defieicny
122
Increased PTT, pt with bleed. Corrects with mixing studies
Factor VIII, IX or XI deficiency~ related to INTRINSIC pathway
VIII = Heme A
IX = Heme B
Hemophelias are X linked but can also be sporadic
123
presents with asymmetric craniofacial physical examination findings, including jaw asymmetry and the appearance that the ear and eye ipsilateral to the injured side are smaller.
Congenital muscular torticollis
| is the most common cause of torticollis in infancy.
124
Cause of head tilt, which presents in the first several months after birth. Symptoms may also include vomiting, irritability, and increased drowsiness. The etiology of is not known, but is thought to be related to migraine physiology. It is self-limited, but can be recurring. Episodes usually last a few hours, and occasionally for several days.
Benign paroxysmal torticollis of infancy (BPTI)
125
Flank mass, thrombocytopenia, and hematuria are the classic features associated with ...
renal vein thrombosis
In the neonatal period, perinatal asphyxia, hypovolemia, sepsis, and vascular thrombosis are risk factors for acute kidney injury.
126
In girls, initiation of the growth spurt (IGS) typically occurs around age _____, with a peak height velocity of _____cm/year achieved at age ____when most girls are at sexual maturity rating (SMR) 3.
9. 5 years
8. 3 cm/yr
11. 5 years
127
Boys have IGS at ___ of age, and reach a peak height velocity of ____ cm/year when they are in genital SMR 3 to 4
11. 5 years
| 9. 5 cm/year
128
______ should be recommended for patients with severe hereditary spherocytosis (hemoglobin level chronically < 11 g/dL).
Splenectomy
129
Splenectomized patients are at higher risk for bacteremia with encapsulated organisms, including...
Patients undergoing elective splenectomy should be...
- pneumococcus, meningococcus, and Haemophilus influenzae type b.
- -vaccinated against encapsulated organisms prior to the splenectomy.
130
A woman with phenylketonuria that is poorly controlled (phenylalanine levels consistently > 360 µmol/L) during pregnancy will be at highest risk for having a child with
intellectual disability, microcephaly, or both. Other associated findings include poor behavioral outcomes, congenital heart defects (8%-12%), and intrauterine growth restriction
131
The bacterial organisms most commonly involved in wound infections arising from dog bites are...
Treatment includes...
Staphylococcus aureus and Pasteurella species
Tx: Amox-clavulanic
Consider Rabies vaccine + IG if there is any concern
132
Pt with polyuria and polydipsia in the context of a craniopharyngioma, a scenario consistent with...
diabetes insipidus (DI). DI occurs when there is a deficiency of antidiuretic hormone from the posterior pituitary.
133
_____ deficiency is the most common anterior pituitary hormone deficiency at the time of diagnosis of craniopharyngioma
Growth hormone
134
is the most common suprasellar tumor seen in childhood. Although it is a benign tumor originating from the remnants of the Rathke pouch, it can cause significant problems because of its location and mass effect.
Craniopharyngioma
| Tx is resection; issue d/t panhypopit, DI, hypothalmic obesity and vision loss
135
Port-wine stains are vascular malformations (capillaries) that are present at birth and remain throughout life and don't proliferate, if located over the distribution of V1 you are at increased risk for...
Sturge Weber Syndrome
Increased risk for seizure/ stroke (from leptomeningeal angiomatosis) glaucoma and ID.
Tx pulsed dye laster as gold standard
136
Patient with ascending paralysis and LE weakness with absent reflexes. Recent camping trip
Tick paralysis... can occur 4-7 days after camping trip
| Do skin exam, remove the tick
137
Pt with unilateral chronic lymphadenopathy otherwise well. Has TB test with 12mm induration, normal CXR.
Most likely bug?
Tx?
Mycobacterium avium complex
| Tx is surgical excision w/o ABx unless sinus tract involvement and NOT I/D
138
For AOM, Antimicrobial drugs should be considered for infants younger than_____, children younger than 24 months with _______, and children of all ages with severe disease
6 months
| bilateral acute otitis media
139
Testing recommended for sexually active women <25 yo
- Annual testing for Chlamydia trachomatis and Neisseria gonorrhoeae using a urine or vaginal swab specimen for nucleic acid amplification testing (NAAT)
- Cervical cancer screening by cervical cytology (ie, the Papanicolaou test) at age 21 years, repeat q3yr til 29
- herpes simplex virus infection, syphilis, trichomoniasis, bacterial vaginosis, hepatitis A, and hepatitis B is NOT advised
140
MSM recommendations
- Annual Screening for HIV/Syphillis
- Annual screening for Chlamydia/Gonorrhea if insertive anal intercourse; urine NAAT
- If practicing receptive anal intercourse: rectal swab NAAT for both organisms
- If practicing receptive oral intercourse: pharyngeal swab NAAT for N gonorrhoeae only (testing for pharyngeal infection with C trachomatis is not recommended)
141
occur in up to 50% of infants with hypernatremic dehydration
Neurologic sequelae
Infants with hypernatremic dehydration have increased sleepiness and can be hyperirritable with a high-pitched cry. Hypertonicity causes brain shrinkage due to neural cell dehydration, increasing the risk for the rupture of bridging veins and leading to subdural, subarachnoid, or intraparenchymal brain hemorrhage. Additionally, patients with hypernatremic dehydration are at increased risk for venous thrombosis.
142
Patient that has intoeing, sits in 'W' position. On exam has 65 degree internal rotation of hip and 40 degree external rotation
Femoral anteversion
will have structually normal hip, have 65 degrees or more of internal hip rotation
--ALL newborns have this
--resolves by 5-6 years of age, reassure
143
Infant normal at birth. Now 18 months with course facial features, cognitive decline and macrocephaly, hearing loss, skeletal dysplasia and short status with hearing loss and HSP.
Corneas are clouded.
Corneas are NOT clouded
Clouded corneas = HURLER (vision affected, Auto-Recessive)
| normal vision = HUNTER (hunter must be able to see X marks spot= X linked~ boys)
144
Pt with intermittent recurring crops of itchy red bumps for past 6 months that get better and leave behind area of hyper pigmented macules and papules. Mostly on extremities.
Dx and cause
Papular uticaria
| From bug bites like fleas, mosquitoes, mites and bedbugs seen during spring/summer
145
is the most common cause of preventable childhood blindness worldwide. It can cause night blindness, Bitot spots, and corneal ulcerations, as well as dry skin, failure to thrive, and increased susceptibility to infection.
Vitamin A deficiency
146
Common presenting features of both polycystic ovary syndrome (PCOS) and nonclassic congenital adrenal hyperplasia (NCCAH) in adolescent females are
hirsutism, menstrual irregularities, and acne. PCOS affects up to 10% of reproductive-age females. NCCAH affects 1 to 2 in 1,000 in the general population.
147
How do you diagnose nonclassic CAH which presents similar to PCOS?
An early-morning, follicular-phase 17-hydroxyprogesterone > 200 ng/dL
An adrenocorticotropic hormone (ACTH) stimulated level higher than 1,000 ng/dL confirms the diagnosis of NCCAH.
