2016 Pearls Flashcards
Which growth chart is preferred for infants under 24 months?
World Health Organization (WHO) growth charts better reflect “ideal growth” of breastfed infants and are superior to US Centers of Disease Control and Prevention growth charts. The WHO charts should be used to assess growth of children younger than 2 years of age.
What is the best assessment of acute undernutrition in a child under 3yo?
For children younger than 3 years of age, the best assessment of acute undernutrition is a weight-for-height below the fifth percentile.
A teen comes to clinic with the following symptoms three weeks after unprotected sex: mucopurulent cervical discharge, intermenstrual bleeding, cervical friability, and lower abdominal pain. Likely Dx?
Recommended test and tx?
Gonococcal Cervicitis
nucleic acid amplification test and treatment with ceftriaxone 250 mg intramuscularly as a single dose and azithromycin 1g orally as a single dose (this is for Chlymadia co-infection)
What is the treatment for gonoccocal cervicitis?
CTX 250mg X1 IM,oral cephalosporins are no longer recommended as first-line medications for treatment of N gonorrhoeae because of the concern for resistance.
What is the treatment for Chlamydia
either azithromycin 1 g orally as a single dose (preferred) or doxycycline 100 mg orally twice a day for 7 days.
Child with small bowel resection presents for lab follow up, he is on TPN + some GJ feeds and is found to have anemia with MCV of 100. What vitamin is he likely deficient?
Vit B12
A 24-hour-old newborn with progressive obtundation, seizures, and tachypnea. Pregnancy, labor, and delivery were uneventful. The newborn initially did well during the first several hours after birth, but then became lethargic, hypothermic, and developed poor feeding. Results of a CMP including glucose, complete blood cell count with differential, and C-reactive protein and Anion gap are all normal with VBG showing respiratory alkalosis… what lab should you get?
Serum Ammonia
classic presentation of a urea cycle disorder, with decompensation in the first 24 to 72 hours of life with progressive respiratory alkalosis, obtundation, and hyperammonemia, in the presence of a normal anion gap.
How do urea cycle disorders present in neonate?
Urea cycle disorders clinically manifest with immediate decompensation in the first 24 to 72 hours of life with progressive respiratory alkalosis, obtundation, and hyperammonemia in the presence of a normal anion gap
What is the inheritance pattern of urea cycle disorders?
All of the urea cycle disorders are autosomal recessive, except OTC, which is transmitted by X-linked recessive inheritence.
New born does well initially then has vomiting, poor feeding and lethargy. Labs show metabolic acidosis, ketosis, hyperammonemia with elevated LFTs and low BG with neutropenia. Likely cause?
Organic acidemias or organic acidurias
Newborn presents with jaundice, HYPOtonia, bruising and bleeding. You obtain CMP showing markedly elevated LFTs and INR is very prolongued. On eye exam you see cataracts. Dx?
Galactosemia
What bacteria are patients with galactosemia at increased risk of getting sepsis from?
What lab abnormalities do you see?
Escherichia coli
Labs: positive urine-reducing substances, abnormal liver function studies, coagulation abnormalities suggestive of a progressive bleeding diathesis, elevated erythrocyte galactose-1-phosphate
Patient presents with recurrent bacterial infections: 5 episodes of AOM, 3 abscess formations, 2 episodes of PNAs. CBC shows normal neutrophil count. What is the gold standard for diagnosis and treatment of this child?
The gold standard for diagnosing and initial management of CGD (innate immune dsfnx) is testing the phagocyte oxidative burst by flow cytometry and initiating prophylaxis with trimethoprim and sulfamethoxazole.
Patient presents with recurrent bacterial infections: 5 episodes of AOM, 3 abscess formations, 2 episodes of PNAs. CBC shows normal neutrophil count. What is the inheritance pattern of this disease
Chronic granulomatous disease is a genetic disorder that can be transmitted by X-linked or autosomal recessive inheritance. The X-linked form is by far the most common
The______ immune system is comprised of barriers such as skin and mucosal membranes, and phagocytes such as the neutrophil, macrophage, and natural killer cells. Dysfnx here results in ____ infections
innate
frequent bacterial or fungal
The _____immune system is comprised of B and T lymphocytes and their subsets; dsynx here results in _____ infections
adaptive
abnormal viral infections
Pain with activity or lumbar extension in adolescents with anatomical cause is _______
You would order this image/study____
Spondylosis (crak in pars interarticularis = posterior aspect of vertebral ring)
Order XRAY~ 50-60% sensitivity
Get MRI if XRAY not conclusive but strong clinical suspision
Autosomal recessive condition with skeletal abnormalities, neutropenia, short stature and exocrine pancreatic insufficiency
Shwachman-diamond syndrome
EPI or exocrine pancreatic insufficiency occurs when lipase secreation <90% resulting in: fat malabsorption, wt loss,FTT, fat-soluble vitamin deficiency. EPI seen in which diseases
Cystic fibrosis chronic pancreatitis Johanson-Blizzard syndrome (HypoT, DD) Pearson syndrome (anemia, organ abnormalities) Schwachman Diamond
How do we treat exocrine pancreatic insufficiency?
PERT (pancreatic enZ replacement Therapy) and supplment fat soluble vit
How many doses of IPV are recommended and at what age?
Minimum interval between doses?
4 total at 2, 4, 6 to 18 months and AFTER 4th birthday (4-6 years)
Minim 4 weeks between 1/2, 2/3
Minimum 6 months between 3/4
If 4th dose BEFORE 4th bday, will need another dose
Who needs an IPV booster if they are fully immunized?
Before travel to polio endemic areas for at least 4 weeks, travelers should get single lifetime booster
Which infants are at increased risk for polycythemia?
Hct >65%
Hypoxic environment; high elevation delevier area, >40 weeks GA, SGA, IDM, mHTN, tobacco exposure
Tri 13, 18, 21
Neonatal Graves, CAH, CCHD, hypoT
Indications for tx polycythemia in newborn?
symptomatic with HcT >60% or Asymptomatic >70%
-tx with partial exchange transfusion
Signs of polycythemia; irritable, tachypnea, hypoglycemia, lethargy, pulm HTN, stroke, seizure, renal vein thrombosis 2/2 hyperviscosity
What diagnostic test should be performed on school age child you suspect has asthma?
Spirometry; gives objective info about airway obstruction… repeat 15 to 20 minutes after administration of inhaled bronchodilator to establish reversible airflow obstruction
Pediatric melanoma often do NOT exhibit typical ABCD detection criteria and often appear:
Pink, red or papular/nodular, have uniform color and smooth borders with “EFG” characteristics
Elevated, Firm, Growing progressively >1 month; may be amelanotic or nodular melanoma
CHARGE stands for:
Inheritance pattern:
Coloboma, Heart defect, choanal Atresia, Retarded growth/devo, Genital anomalies, Ear anomalies
Autosomal DOMINANT
Define Kocher Criteria
5 characteristics for Septic Arthritis (pyogenic arthritis) of hip
- T >38.5
- WBC >12,000
- ESR >40
- Inability to ambulate
- CRP > 2.5 mg/L
* Higher inflammatory markers more likely pyogenic vs transient synovitis
Commonly implicated organisms in pyogenic arthritis (septic arthritis)
Staph Aureus, Streptococcal species
Kingella in kids <2yo
Athletes are considered functionally 1-eyed if corrected vision is worse than ____ in one eye.
What are high risk eye injury sports
< 20/40
Baseball, basketball, hockey and lacrosse
Infant presenting with feeding difficulties, liver damage, cataracts and signs of shock liver
Concern for galactosemia; inborn error metabolism
Infant with hypoglycemia, liver failure/dx, poor growth starting around age 6 months, previously exclusively breast fed
Concern for Fructose intolerance
____ is teh absence of the iris and associated with WAGR syndrome
Aniridia
Wilms tumor, Aniridia, Genital anomolies, Retardation
____ is devo defect of eye, one or more ocular structures are missing and associated with which syndrome?
Coloboma CHARGE (Coloboma, Heart defect, choanal Atresia, growth Retardation, Genital anomalies, Ear anomalies)
___ optic neuropathy associated with elevated intraoccular pressure
Glaucoma
primary congenital, infantile, juvenile are rare
Secondary glaucoma from Sturge-Weber, aniridia and ROP can occur
Metabolic dx associated with cataract
Galactosemia, hypoPTH, hypoG, DM, intrauterin infections (TORCH) or genetics such as trisomy 13, 18, 21
RF of low BW, IDM, ocular trauma or glucocorticoids in utero
Patient presents with swelling of face and arms, cough, difficulty breathing while laying down
concern for Superior Vena Cava compression
- often 2/2 to mass
- Concern for maintaining patency of RA; you can collapse RA by negative intrathoracic pressure, gravity (laying supine or sedation avoided!)
Child comes in with tripodding, drooling and thumb sign on lateral neck xray.
Dx____
Would you do antiBx, IM dex, 100% oxygen first to stablize pt?
Epiglottitis
Give oxygen and immediately consult someone to establish a stabalized airway
Dex is not shown to improve outcomes. AntBx will be necessary but do NOTHING to aggitate the patient until airway stablized; <5yo will be intubation in all cases of epiglottitis
_______ or reduced visual acuity due to abnormal visual development early in life, is the most common cause of visual impairment in children. It can be caused by strabismus, refractive errors that differ significantly between the 2 eyes, or by interruption of the visual axis.
Amblyopia
Although it is normal for infants to have a disconjugate gaze in the first 2 to 4 months after birth, eye movements should be conjugate by
6 months
______-associated epidemic keratoconjunctivitis is very contagious; infection can result in hospital and community outbreaks
Adenovirus
Epidemic keratoconjunctivitis can occur after direct contact with infected health care workers or contaminated equipment during eye examinations.
______ sequence describes pulmonary hypoplasia, growth restriction, compressed facies, and abnormal positioning of the hands and feet in neonates born to mothers with low amniotic fluid levels
Oligohydramnios
The extent of pulmonary hypoplasia in oligo/Potter is not proportional to
Neonates with pulmonary hypoplasia also may have _____
amniotic fluid levels in utero.
pulmonary hypertension
In cases where the history is suggestive of exercise-induced asthma but there is not a good response to pretreatment with albuterol, the next most likely diagnoses are …..
VCD and anxiety, which manifest similar symptoms
______ are firm, noncompressible, slowly growing nodules located most often on or near the lateral eyebrow.
Lesions located in the midline (glabella, occipital scalp, midline back) or midline dermal sinuses require imaging to assess for central nervous system extension.
Dermoid cysts
Evaluation of thyroid nodules includes
a thyroid-stimulating hormone level, thyroid ultrasonography, and referral to a subspecialist experienced in the evaluation and management of pediatric thyroid nodules.
Thyroid nodules of_____ or greater warrant ultrasonography-guided fine needle aspiration.
1 cm
cancer is associated with the RET proto-oncogene and inherited in an autosomal dominant manner. Nodules that are firm, irregular, or fixed or that show microcalcifications or irregular margins on ultrasonography confer increased risk for malignancy.
Medullary thyroid
Thyroid cancer occurs in about_____of thyroid nodules in children and adolescents, vs about 5% in adults.
_______ the most common thyroid cancer type in both children and adults, is more aggressive in childhood than adulthood but survival is better.
25%
Papillary thyroid carcinoma
is an autosomal dominant connective tissue disorder with variable expression among members of the same family. Classic manifestations include ocular, cardiovascular, and skeletal involvement.
Marfan syndrome
Marfan syndrome, caused by mutations in the _____is clinically diagnosed based on family history and characteristic systemic findings.
FBN1 gene
In patients with Marfan syndrome, surveillance consists of
an annual comprehensive physical and an ophthalmologic (including slit-lamp) examination, and echocardiography
Contraindications to pertussis vaccine include
anaphylaxis following a previous vaccination or development of encephalopathy that cannot be ascribed to another reason in the 7-day period following pertussis vaccination. That said, there is no evidence that links pertussis vaccination with any acute-onset neurologic illness.
is felt to be largely responsible for the resurgence in cases of pertussis in the United States in the last few decades.
Waning immunity
Immunity to pertussis wanes over a period of 7 to 20 years after vaccination. Several epidemics in the United States have revealed high attack rates in the 7- to 10-year-old and 10- to 14-year-old age groups, coinciding with the expected timeframe for waning from the last dose of DTaP in the childhood series (4 to 6 years)
The primary treatment of children with posttraumatic stress disorder is
evidence-based trauma-focused psychotherapy, which involves a therapist working with both parent and child.
is diagnosed in children and adolescents who have experienced actual or threatened harm to themselves or others and who manifest symptoms of intrusive and uncontrollable thoughts, avoidance and distress with reminders of the trauma, and altered cognitions and mood for at least a month with resultant impairment in functioning.
Posttraumatic stress disorder
The Langley Model of Improvement is a framework for quality improvement that addresses 3 questions:
What are we trying to accomplish?
How will we know that a change is an improvement?
What changes can we make that will lead to an improvement using multiple, rapid Plan-Do-Study-Act cycles.
