2018 Pearls Flashcards
Contraindications to rotavirus vaccine administration are…
Infants with a personal history of intussusception or a personal history of an anaphylactic reaction to the rotavirus vaccine should not receive the vaccine.
There are age limits beyond which the Rota vaccine should not be administered
(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
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 .
irradiated
Sx: skin (rash) elevated LFT/bili, diarrhea
Irradiation of blood products
damages the DNA of donor lymphocytes, thereby rendering them replication incompetent and definitively preventing transfusion-associated graft-vs-host disease.
____ 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.
Motor tics
Simple motor tics are brief, individual movements such as shoulder twitching, forceful eye blinking, or eye rolling.
The presence of hepatitis B surface antigen and total antibody to hepatitis B core antigen without IgM hepatitis B core antibody is indicative of
chronic hepatitis B virus infection.
The presence of _____ suggests high viral replication and increased risk of hepatitis B virus transmission.
hepatitis B e antigen
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
topical sirolimus.
In primary adrenal insufficiency, there is a deficiency of both glucocorticoid (cortisol) and mineralocorticoid (aldosterone). In response to low cortisol levels, _____ levels are high.
adrenocorticotropic hormone (ACTH) this causes hyperpigmented skin
Fatigue, nausea, weight loss, hypotension, volume depletion, and diffuse hyperpigmentation are common presenting symptoms and signs of ____
What lab findings do you expect?
Addison disease hypONa hypERK hypOglycemia metabolic acidosis low cortisol high ACTH eosinophelia
First line medication for depression in teens
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
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?
Glycogen storage disease type 1
Autosomal recessive
In glycogen storage disease type 1 what labs abnormalities do you see?
hypoglycemia, lactic acidosis, hyperlipidemia, hypertriglyceridemia, and hyperuricemia.
autosomal recessive lysosomal storage disorder that has a spectrum of presentations but universally includes bone disease, hepatosplenomegaly, cytopenias, and pulmonary disease.
Gaucher disease
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.
Hurler syndrome
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
Peroxisomal biogenesis disorders
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?
Pompe
–elevated creatine kinase level and urinary oligosaccharides
Children with _______ have recurrent episodes of hypoglycemia, lactic acidosis, hyperlipidemia, hypertriglyceridemia, and hyperuricemia.
glycogen storage disease
Pediatric hypertension is defined as the sustained elevation of systolic or diastolic blood pressure
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
Children who are symptomatic or who have secondary hypertension, ventricular hypertrophy, hypertensive retinopathy, or diabetes mellitus should be
started on antihypertensive medications in addition to lifestyle modifications.
When do we start ABx on burn patients?
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.
What are some expected changes in vitals in burn patients?
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.
Kiddo who is a mouth breather, mucoid nasal discharge and on exam has grape like lesions in BL nares
ML diagnosis
Nasal polyps
CF… this is the MCC of nasal polyposis in children
______ 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
Enterovirus
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
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
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)
Signs and symptoms of_____ deficiency may include hyporeflexia, ataxia, decreased vibratory sensation and proprioception, muscle weakness, and limitation of eye movement
vitamin E
Congenital neutropenia PLUS
short limbs, dwarfishm, fine hair, shitty immune system
Cartilage-hair hypoplasia
Congenital neutropenia PLUS varying degrees of oculocutaneous albinims
Chediak Higashi
Congenital neutropenia PLUS FTT, exocrine pancreatic dysfunction (diarrhea) and skeletal anomalies
Shwachman-Diamond syndrome
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
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
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
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.
Children between the ages of about 1 and 5 years are especially susceptible to “toddler fractures,” which are
oblique fractures of the distal tibia.
A loud systolic murmur at the right upper sternal border and associated with a click best fits a diagnosis of
aortic stenosis
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
abrupt onset of pharyngitis, palpebral conjunctivitis, fever, moderate degree of illness, and preauricular lymphadenopathy.
Adenovirus
pharyngitis with vesicles or ulcers; exudate is less commonly seen. A variety of exanthems and enanthems are associated
Coxsackievirus and echovirus
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
_______ 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
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.
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
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
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.
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
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
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
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.
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)
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
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.
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.
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
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.
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
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.
