PBR Flashcards
What is the normal age range for pubertal development?
9-13yo for both boys and girls
How long does it take to go from Tanner stage 2-5?
5 years
True or False.
Adolescent peak height velocity has more to do with Tanner stage than age.
TRUE
I have no pubic hair, a childlike penis and my testicles are < 2.5ml. What is my SMR?
1
I have fine pubic hair and wrinkles on my scrotum. What is my SMR?
2
I have coarse, curly pubic hair and my scrotum and penis have grown. What is my SMR?
3
I have dense, curly pubic hair and a full size penis, what distinguishes me between SMR 4 and 5.
SMR 5 has adult size scrotum, penis and testicles and hair is also on inner thigh.
I have fine hair along my labia and breast buds. What is my SMR?
2
I am flat chested and have no pubic hair. What is my SMR?
1, unless I am a supermodel waif who has been waxed.
My breasts go beyond the areola and my pubic hair is coarse and curly. What is my SMR?
3
My SMR is 4. What is my pubic hair and breast situation?
Pubic hair is dense, coarse and curly but not extending to thigh. Breasts have a secondary mound and papilla now.
My SMR is 5.What is my pubic hair and breast situation?
Hair extends to inner thigh. Areola is no longer a separate projection from the breast.
What is classified as delayed puberty?
No development by 14 for a boy and 13 for a girl.
What is the pre-puberty height velocity?
What is the pre-adolescent height velocity?
5-6 cm/year
9-10 cm/year
When does growth spurt begin?
At breast development for girls and at testicular enlargement for boys.
A 13yo girl is found to have an elevated alkaline phosphatase on routine screen. What is your next step?
Reassurance.
What is bone age vs. chronological age in constitutional growth delay?
Bone age < Chronological age. The bones catch up later
True or False.
Boys reach SMR 4 prior to peak height velocity.
TRUE
True or False.
HCT increases with growth spurt.
True.
What is the sequence of male pubertal development?
Testicular growth then pubarche then penile growth then peak height velocity
Ball then bat then height.
What is the most common cause of delayed puberty in boys?
Constitutional delay
At what age is pubertal development considered too early? What is normal onset?
Age 9 is too early. Age 10-11 is normal
When do boys develop axillary hair?
After SMR 5.
A boy has pubic hair development and penis enlargement but no testicular gland enlargement. What does this mean?
Androgen stimulation is coming from outside the gonadal area.
What is the difference in pubic hair development between boys and girls?
Pubarche occurs 1 year after testicular growth in boys vs. girls where it occurs parallel to breast development.
A 13yo complains of breast development with milk from his breasts. He is otherwise healthy but does smoke marijuana. What do you advise?
Stop the marijuana.
What is the sequence of puberty in girls?
Breast bud then Pubarche then Peak Height Velocity then Menarche
What is too early puberty for girls?
Younger than 8yo is too early.
What is the first sign of puberty in girls? What age does it occur? By when should it occur?
Thelarche (breast tissue development). Usually occurs at age 11, but should occur by age 13.
Which hormone(s) are responisible for breast and pubic hair development in girls?
Estrogen for breast and Androgen for pubic hair
How long after thelarche do you expect menarche?
2 years
In which SMR does a girl have peak height velocity?
SMR 3 or 4.
How far from adult height are girls at menarche?
2 inches.
An 11yo girl who is in SMR 2 has bloody vaginal discharge. What do you do?
Look for vaginal foreign body, such as toilet paper.
True or False.
If thelarche occurs without pubarche, think androgen insensitivity.
TRUE
True or False.
If Pubarche occurs without thelarche, think excess androgen/low estrogen.
TRUE
A 3yo has developed breast buds. Her mother is very worried. What is your response?
This is okay, as long as there is no further development and there is no pubarche. This may be due to obesity.
Which of the following kids is undergoing precocious puberty? Pick all that apply.
A) A 9yo girl who has started menarche with breasts and pubic hair.
B) A 10 yo boy who has developed pubic hair and genitals.
C) A 7yo with thelarche and pubarche but no menarche
D) A 9yo with thelarche and pubarche but no menarche
E) A 7yo girl with thelarche but no pubarche and no menarche
A) yes B) no C) yes D) no E) no
What is premature pubarche without thelarche due to?
Adrenal androgen production
Which hormone is responsible for axillary hair and acne?
Androgen
You evaluating a girl for precocious puberty and note that she also has visual field defects. What is the likely cause of precocious puberty?
CNS lesion…pituitary
You are evaluating a boy for precocious puberty and not acne, facial hair and muscle bulk. What is the cause?
Excess androgen from outside of the gonads.
A prominant labia minora or a change in vaginal color is due to which hormone?
estrogen
A girl has premature breast development. What is your test of choice?
Ovarian ultrasound
A boy has premature pubarche what is your test of choice?
Adrenal ultrasound
How do you differentiate peripheral vs. central causes of precocious puberty?
LH, FSH and adrenal steroids and MRI.
What is the most likely reason not to use contraception?
Desire for pregnancy
True or false.
50% of all pregnancies occur within 6 months of first time experiencing intercourse and 20% occur in 1st month.
TRUE
What are absolute contraindications to OCP?
Breast Cancer CAD or Cerebrovascular Disease Hepatic Disease Elevated Lipids Pregnancy
Of the following patients who would you not give OCPs to? A) A sickle cell patient B) A epilepsy patient C) A girl whose mother has breast cancer D) A girl with lymphoma E) A girl with hyperlipidemia
E.
How often do you screen for HPV?
If Sexually active, every year.
If not sexually active, start at age 18 then repeat every 3 years.
When do you start workup for amenorrhea?
No period by age 16
No thelarche or pubarche or menarche by age 14.
No period for 3 months in a 6 month span.
What are the expected Lab findings in PCOS?
LH:FSH ratio > 2.5 and elevated androgen level.
What is the treatment for PCOS?
OCP, weight loss, and anti-androgen medications like spironolactone
A 13yo girl presents with amenorrhea, your partner saw her and dismissed her 6 months ago without doing a pelvic exam, but you notice she does not have any pubic hair despite having developed breasts. What is the likely problem?
Androgen insensitivity
An amenorrheic 13yo girl has breast buds but no pubic hair. What should be your next step?
Karyotype for Turner’s syndrome
A 15yo marathon runner has not had a period in 6 months. What do you expect on her labs? What is she also at risk for?
Low estradiol. Osteoperosis and Anorexia nervosa.
Does resumption of menses reduce the risk of osteoperosis in a girl with stress induced amenorrhea?
yes
What is the pathophysiology of primary dysmenorrhea? How do you treat it?
Excess prostaglandins. Treat with NSAIDs
True or False.
Primary dysmenorrhea is a significant cause of school absence.
TRUE
True or False.
Exercise, healthy diet and tylenol may help in reducing primary dysmenorrhea.
False.
How do you distinguish endometriosis from primary dysmenorrhea?
Endometriosis pain occurs any time in cycle, not just during menses.
A teenager presents with recurrent nightmares, fear of being alone, diminished appetite, and diminished interest in school. She is otherwise healthy but was raped on campus last year and has been depressed since then. What is her diagnosis?
PTSD
What is the leading cause of death among teenagers?
Accidents (MVA)
Homicide
Suicide
Which of the following is not a requirement to diagnose anorexia nervosa?
A) Distorted body perception
B) Poor caloric intake
C) Intense fear of weight gain
D) Weight below 15% expected
E) Absence of 3 consecutive menstrual cycles.
B.
Which of the following is required to diagnose anorexia nervosa? A) Depression B) Excess exercise C) Poor caloric intake D) Distorted body perception E) Diuretic abuse
D.
Which is not an indication for inpatient admission in a patient with bulemia nervosa? A) Failure of outpatient treatment B) Dehydration C) ECG abnormalities D) K+ of 3.0 E) Suicide ideation
D
How do you distinguish achalasia from bulemia?
Achalasia is involuntary
What is the metabolic risk in refeeding an anorexic or bulemic patient?
Hypophosphatemia
What is the most prevalent STD among teens?
HPV
What is the most common bacterial STD?
Chlamydia
True or False.
Genital warts caused by the HPV is the most prevalent STD.
False. Even though HPV is most transmitted STD, genital warts are not.
A young mother is devastated that her daughter has been diagnosed with gardenerella vaginalis and thinks her 12yo daughter has been sexually active. The girl swears she has not been. How do you explain the infection?
This is BV. It can be passed non-sexually even from toilet seats and door knobs.
You do a pelvic exam on a 15yo sexually active woman. You quickly note a fishy odor on applying KOH to the smear. In addition, you note clue cells on the smear. What is your diagnosis?
BV
True or False.
Males do not get trichomoniasis.
False. They can get it, but are asymptomatic usually.
A 15yo girl complains of vaginal itching and burning along with dyspareunia. On exam you not a frothy yellow discharge with a strawberry cervix. What do you expect on wet mount?
Flagellated organisms.
What is the treatment for trichomoniasis? Do you need to treat the partner?
Treat with 2 grams metronidazole. Treat the partner too.
True or false.
Gonorrhea is usually asymptomatic.
True.
What is the major complication of untreated GC in men and in women?
Men can lead to severe epididymitis. Women can get peri-hepatitis, AKA Fitz-Hugh Curtis.
What are symptoms of disseminated gonococcal infection?
Rash, arthritis, possible meningitis and endocarditis.
What is the treatment for genital herpes ulcers?
7 days po acyclovir.
An asymptomatic sexually active 15yo has now changed her sexual partner since her last exam. What is the most appropriate study at this time?
Urine PCR for chamydia and gonorrhea.
What are the diagnostic criteria for PID?
Lower abdominal or Pelvic pain AND EITHER
Uterine or Adenexal tenderness
OR
Cervical motion tenderness.
Which of the following tests are required when doing a pelvic exam and drawing blood for PID evaluation? A) Chlamydia culture B) GC culture C) Pap smear D) Vaginal swab for Trichomoniasis E) Vaginal swab for BV F) RPR G) HIV
All of them. And you must counsel on the HPV vaccine.
What is the outpatient treatment regimen for PID?
Azithromycin 1gram x 1 and Ceftriaxone 250mg IM x 1
What do you do if pain persists despite outpatient treatment?
Do U/S to look for TOA and admit.
When do you expect resolution of RUQ pain upon treatment of Fitz-Hugh Curtis?
Within 2 days.
A 15yo girl comes in with fever, RUQ pain that radiates to her shoulder, and nausea. Her abdominal ultrasound is normal. She is otherwise healthy and her only medication is an OCP. What is your next step? A) ERCP B) Abdominal CT C) Pelvic exam D) Surgical consult E) Serum amylase and lipase
C.
Which of the following require parental informed consent? A) Life threatening emergency B) Medical Care during pregnancy C) Blood Donation D) Substance Abuse Treatment E) STD treatment F) HIV testing G) Sexual assault management H) Any care for an emancipated minor
C.
? Whats inside? Serous edemal fluid :)
? Vaguely demarcated area of edema found
in presenting part of the scalp
? Associated with:
o Molding of head
o Overriding of parietal bones
? Features: o External to periosteum o Crosses suture lines and midline o Overlying skin with petechiae or purpura
? No specific treatment
? Resolves in 48-72 hrs
Caput Succedaneum
Whats inside? Blood :)
? Subperiosteal collection of blood
? Associated with: o Rupture of capillaries traversing from skull to periosteum o Difficult labor and delivery ? Features: o No extension across suture lines o Parietal bones involved mostly o Overlying scalp not discolored o More apparent after several hours or days
? No therapy for uncomplicated lesions
? Most common complication:
Hyperbilirubinemia
cephalhematoma
Collection of blood beneath the aponeurosis
? May extend from eyes to nape
? Firm fluctuant mass increasing in size after
birth
? Mechanism of injury: rupture of emissary
vein
? Severe anemia and hypovolemic shock
? Volume replacement and blood products
? Resolves over 2-3 wks
? High morbidity and mortality
Subgaleal Hemorrhage
Uncommon in neonates because: o Skull bones are less mineralized thus are compressible o Bones separated by membranous sutures (allows molding)
? Features: o Associated with forceps delivery o Most fractures are linear ? This is good because they do not require treatment o Depressed fractures are rare o Entirely asymptomatic unless with associated intracranial injury
skull fractures
Diagnosed by X-ray
? For linear fractures ? No treatment
? For depressed fractures with neurological
signs? immediate surgical intervention
skull fractures
Most serious brain lesion in the neonatal
period
? Major cause of death
? Incidence increases with decrease in
birthweight & AOG
? Results from trauma, asphyxia or primary
hemorrhagic disorder
Periventricular- interventricular hemorrhage
Bleeding site: subependymal germinal matrix o Area where future neurons & glial cells will migrate from o Very vascular o Very thick in young/preterm babies
Periventricular- interventricular hemorrhage
Predisposing factors: hypoxic-ischemic
injury, pneumothorax, hypovolemia,
hypotension
? 80-90% occur w/in the first 2 postnatal days
? Clinical manifestations: apnea, lethargy, poor
muscle tone, convulsions and bulging
fontanel
Periventricular- interventricular hemorrhage
Diagnosis: cranial US
? Complications: periventrucular
leukomalacia (PVL), posthemorrhagic
hydrocephalus
Periventricular- interventricular hemorrhage
- Principal ischemic brain lesion of premature
infants - Necrosis of periventricular white matter
- Anterior and posterior periventricular regions
most commonly affected - Dx by US: see cystic changes
- MRI more sensitive or evaluating extent
periventrucular
leukomalacia (PVL)
Major complication of severe IVH
- Progressive ventricular dilatation
- Plugging of arachnoid villi by intraventricular
blood clots
- Cranial US: procedure of choice for dx
- Clinical signs: enlarging head circ, apnea,
lethargy, bulging fontanel or widely split sutures
- 3-5% will require shunt insertion
Posthemorrhagic Hydrocephalus
? Etiology: a. Peripheral paralysis? compression of peripheral part of facial nerve b. Central paralysis ? contralateral CNS injury ? Paralysis may not be apparent at birth ? Type and distribution of paralysis depends on etiology ? No specific therapy ? Most resolve spontaneously
facial nerve palsy
Very common finding
? Bright red patches on bulbar conjunctiva
? Noted in both difficult and easy delivery
? Treatment: reassure parents
? Blood absorbed within 1-2 wks
Subconjunctival hemorrhage
Most frequently fractured bone ? Found in difficult delivery of shoulders ? Greenstick type fracture commonly ? Usually asymptomatic ? Decreased movement of arm on affected side ? Crepitus on palpation ? Irregularities along clavicle ? Callus formation at 7-10 days ? X-ray/radiographs confirm diagnosis ? Therapy is directed at minimizing pain
Clavicle fracture
Paralysis of muscles of the extremities ? Follows mechanical trauma to the brachial plexus (spinal roots of the 5 the cervical to 1st thoracic nerves)
brachial palsy
Most common o Injury to 5-6 th cervical nerves o Arm is adducted and internally rotated, forearm pronated, wrist flexed o Absent Moro on affected side o Suspect when arms are extended because normally they should be flexed o Tx: partial immobilization and proper positioning (to prevent contractures & minimize pain) o Good prognosis arm is extended
Erb-Duchenne/ Upper Arm Paralysis
o Injury: 8 th cervical and 1st thoracic roots o Extremely rare o Paralyzed hand o No grasp reflex o Horner Syndrome (ptosis, miosis, enopthalmos) o Relatively poor prognosis
Klumpkes/ Lower Arm Paralysis
Avulsion of 3rd ,4th ,and 5 th cervical roots which supply phrenic nerve ? Occurs in difficult breech delivery ? Apparent respiratory distress ? X-ray shows elevation of affected hemidiaphragm
Phrenic nerve palsy/ Diaphragmatic paralysis
Affected side cannot contract so it
remains elevated (more than 1
vertebral space)
? Dx: Real time sonography (to rule out
congenital diaphragmatic hernia)
? Spontaneous and complete recovery
Phrenic nerve palsy/ Diaphragmatic paralysis
Uncommon
? Suspected in any newborn with pallor,
irritability and abdominal distension without
evidence of external blood loss
? Liver =most frequently injured abdominal
organ
? Spleen rupture, adrenal hemorrhage, kidney
injuries rare
INTRAABDOMINAL ORGAN INJURIES
- Evidence of fetal distress and all of the following:
1. Profound acidemia (pH5 minutes
3. Evidence of neurological sequelae (seizures, coma,
hypotonia)
4. Multisystem organ failure (CV, GI, hematolog
Criteria for Diagnosis of Perinatal Asphyxia
- Impairment of maternal oxygenation
- Dec blood flow from mother to placenta
- Dec blood flow from placenta to fetus
- Impaired gas exchange across the placenta
- Inc fetal oxygen requirement
Factors That Increase the Risk of Perinatal Asphyxia
Conditions that Predispose to Perinatal Asphyxia
Maternal, Obstetric, Fetal, or Intrapartum?
Hypertension DM CVD Infectious disease Renal disease Drug addiction Anemia/ isoimmunization Age 40
Maternal
Conditions that Predispose to Perinatal Asphyxia
Maternal, Obstetric, Fetal, or Intrapartum?
Abruptio Cord prolapse Placenta previa PROM Hydramnios Multiple gestation
Obstetric
Conditions that Predispose to Perinatal Asphyxia
Maternal, Obstetric, Fetal, or Intrapartum?
Abnormal presentation Precipitous delivery Prolonged labor Difficult assisted delivery Chorio - amnionitis Use of general anesthesia
Intrapartum
Conditions that Predispose to Perinatal Asphyxia
Maternal, Obstetric, Fetal, or Intrapartum?
Prematurity SGA IUGR Post-term gestation Congenital malformation Abnormal heart rate pattern or deceleration
Fetal
An abnormal neurobehavioral state in which the
predominant pathogenic mechanism is impaired
cerebral blood flow
- 20-30% mortality
- 30-50% of survivors develop permanent
neurodevelopmental abnormalities (cerebral palsy;
mental retardation)
Hypoxic-Ischemic Encephalopathy (HIE)
vesicles measure
<5 mm in diameter
bullae measure
> 5 mm in diameter
contain edematous or lymphatic fluid, serum proteins, antigen-antibody complexes, and soluble inflammatory mediators.
Vesicular, bullous, and pustular rash
Infectious Causes in Children (Viruses)
Varicella-zoster virus (wild and vaccine strains)Herpes simplex virus 1, 2; Coxsackievirus A4, A5, A7-10, A16 and B1-3, B5 Echovirus 4, 6, 9, 11, 17, 19, 33 and Enteroviruses 7,2
Infectious Causes in Children (Bacteria)
S. aureus (impetigo, SSS), S. pyogenes (blistering distal dactylitis, ecthyma,erysipelas, impetigo, SSS), Reiters disease
Noninfectious Causes of Vesicles and Bullae In Children, Neonates and Infants
Drug reactions , Stevens-Johnson syndrome , Arthropod bites (Cimex spp. [bedbug], S.scabiei) Contact dermatitis , SLE, Thermal injury, Toxic epidermal necrolysis (Lyell disease)
Primary infection Common highly contagious disease
In normal immunocompetent children,
systemic symptoms usually mild,
serious complications unusual
Varicella (Chicken Pox )
Adults and in children with CMI deficiencies:
constitutional symptoms more severe, eruptions
extensive, occasionally complicated by
pneumonia
Varicella (Chicken Pox )
Mode of transmission: respiratory route or direct
contact with skin lesions of varicella/herpes
zoster patients
Incubation period: 10 21 days
Varicella (Chicken Pox)
characterized by short or absent prodromal period
Prodromal symptoms: fever, malaise, anorexia,
headache, and occasionally abdominal pain for
24-48 hours before appearance of rash.
varicella Chicken pox
Vesicles with irregular margin of erythema that
resemble dewdrops on a rose petal
Varicella Chicken pox
Most striking manifestation of lesion: rapidity of
progression from macule to papule to vesicle with clouding and umbilication within 24 to 48 hours and ends in crusting
Varicella (Chicken Pox)
Lesions appear in crops, generally involve
trunk, scalp, face and extremities
distribution typically central spread peripherally
rash more profuse on the proximal parts of the extremities (upper arms and thighs)
Varicella Chicken Pox
Mild illness with few vesicles Full-blown Varicella syndrome Completely asymptomatic Severe disease with complications in neonates, adults & immunocompromised (Disseminated/Progressive Varicella)
Primary VZV Infection clinical spectrum
Occur in ~ 5% of otherwise healthy subjects
Skin infections
Can cause scarring
Risk of septicemia
Pneumonia
Primary viral or secondary bacterial
Rare in children, more common in adults especially smokers and immunocompromised
Complications of Varicella Chicken Pox
Encephalitis/meningitis Hepatitis Glomerulonephritis Thrombocytopenia Arthritis Reyes syndrome aspirin
Other complications of Varicella Chicken Pox
associated with fever and mild constitutional symptoms
Papulovesicular eruptions
Rapid progression of macules to papules to vesicles and finally to crusts
Appearance of lesions simultaneously in one anatomic area
Predominant central distribution of lesions including the scalp
Eventual crusting of all skin lesions
Clinical Diagnosis
Risk Factors for Progressive Varicella
Malignancy Corticosteroid therapy Pregnant Immunodeficiency Newborns Adolescents and Adults
Neonatal Syndromes resulting from Maternal Varicella
- Congenital defects secondary to intrauterine VZV infection
- Neonatal Chickenpox
- Zoster in infants
Most of the stigmata are due to virus induced injury to the nervous system Cicatricial skin lesions- dermatomal Ocular abnormalities Cataracts Chorioretinitis Horners syndrome Microphthalmia Nystagmus Hypoplastic limbs Cortical atrophy or mental retardation Early death
Congenital (fetal) Varicella Syndrome
17% chance of acute
infection, 31% untreated case mortality rate
5 days before
delivery to 2 days
after delivery
20 days until 6 days
before delivery
Serologic evidence or minimal symptoms of chickenpox
May develop Herpes zoster during infancy
2nd and 3rd trimester up
to 21 days before
delivery
Congenital Varicella Syndrome (2% risk)
1st trimester
Mild disease, or Severe Systemic Infection Fever Poor feeding Pneumonia Vomiting and respiratory distress Mortality 30%
Neonatal Varicella
Varicella zoster virus (VZV) establishes latent infection
in dorsal root ganglia and reactivate
Rare in children younger than 10 years
Incidence higher among children with prior VZV
infection in utero or during the first year of life and
esp. immunocompromised
Herpes Zoster (Shingles)
Tx for Neonatal Varicella
VZIG and Acyclovir
Tx for Herpes zoster:
Acyclovir (oral) 20 mg/k/dose max 800 tid to shorten the course (optional)
Primary varicella:
Option of no treatment in a healthy child unless with complications then treatment with IV acyclovir at 500mg/m2 or 10 mg/k/dose every 8 hours for 7 days
Prevention
Varicella vaccine live attenuated vaccine given in 2 doses ( 12-18 months and at 4-6 yrs)
? Varicella vaccine within 3 to 5 days from exposure
? VZIG within 96 hours from exposure (recommended for immunocompromised children, pregnant women, and exposed newborns)
? Pooled IVIG within 96 hours from exposure
Post Exposure Prophylaxis Options
Family Alphaherpesviridae subfamily herpesvirus
hominis
Herpes Simplex
associated chiefly
with infections of the mouth, lips, eyes and central
nervous system (above the waist)
HSV 1
associated with
genital and neonatal infections
HSV 2
HSV-2, the site of latency is the
sacral ganglia
Viral infection begins at a cutaneous portal of entry such as oral cavity, genital mucosa, conjunctiva or breaks in keratinized epithelia.
HSV
? Various stimuli precipitate recurrent infection:
? manipulation of nerve roots,
? direct trauma to the ganglia,
? exposure to UV light,
? hormonal changes e.g. menses,
? administration of immunosuppressive agents
? and significant intercurrent infection
HSV
? Leading cause of non-epidemic, spporadic encephalitis in children and adults.
? It is an acute necrotizing infection involving the frontal and or temporal and limbic system .
HSV Encephalitis
? Beyond the neonatal period it is almost always caused by HSV 1.
? Symptoms are non-specific but focal signs are common. If untreated it can progress to coma and death in 75% of cases.
HSV Encephalitis
HSV-1, the site of latency is the
trigeminal ganglia
Diagnosis of neonatal HSV should be considered in
babies with S/S of ____
when no bacterial etiology can be identified
bacterial sepsis and/or meningitis
Neonatal infection (evident by 4 to 5 weeks of age) manifest as any of 3 patterns
- skin/eye/mucous membrane infection (SEM)
- CNS infection ( encephalitis) +/- SEM
- Disseminated infection
Congenital infection due to intrauterine exposure
to HSV is (TORCHES)
uncommon
Manifestations of congenital infection include:
skin lesions and scars chorioretinitis
microcephaly hydrancephaly
microphthalmia
Picornaviridae family
Very common viruses with a worldwide distribution
Produce a broad range of important illnesses
The genus name reflects the importance of the GI tract as a primary site of viral invasion and replication and transmission.
Nonpolio Enteroviruses
Hand, Foot and Mouth Disease
Enteroviruses
Most commonly caused by Coxsackievirus A16
The oropharynx is inflammed and contains scattered vesicles on the tongue, buccal mucosa, posterior pharynx, palate, gingiva and/or lips .
Hand Foot Mouth disease
Maculopapular/ vesicular lesions appear in the hands/fingers, feet. Lesions are tender and are common on the dorsal surfaces but can also occur on the palms and soles.
Hand Foot Mouth disease
HFMD caused by Enterovirus ___ is more severe with increased rates of neurologic disease.
71
Sudden onset of fever, sore throat, dysphagia
Characteristic lesions present on the anterior tonsillar pillars, soft palate, uvula and posterior pharyngeal wall occasionally posterior buccal mucosa
Herpangina
Discrete vesicles that ulcerate surrounded by erythematous rings
Resolves in 3 to 7 days
Herpangina
Caused by double -stranded DNA pox virus
Acquired by direct contact or fomites
Mostly affects children 2-6 years
Incubation: > 2 weeks
Molluscum Contagiosum
Discrete, pearly, skin colored, dome shaped papules 1-5 mm with a central umbilication
Self limited disease6-9 months
Treatment of choice: curettage
Molluscum Contagiosum
Erythematous macules that very rapidly evolve into
thin-walled vesicles and pustules.
The vesiculopustular stage is brief and following
rupture, sticky, heaped-up, honey-colored crusts
are formed
Impetigo contagiosa
Risk factors: Insect bites, scabies, cutaneous injuries, and preceding dermatitis serve as portals of entry for the organism, which does not penetrate intact skin.
Impetigo contagiosa
Caused by group A ? hemolytic streptococcus or
staphylococcus aureus
Impetigo contagiosa
____ often caused by S. aureus, most
often phage group 2
Mainly an infection of infants and young children
Bullous impetigo
Disease of infants and young children under 10 years
Caused by group 2 phage type S. aureus
Clinical manifestations due to elaboration of exfoliative
(epidermolytic) toxin by the infecting organism (ETA or
ETB)
Staphylococcal Scalded Skin Syndrome (SSSS)
Onset of rash preceded by prodrome of malaise, fever,
and irritability associated with exquisite tenderness of
the skin or the appearance of generalized erythema
may be abrupt without preceding symptoms
Initially, the eruption is macular and involves the face,
neck, axilla, and groin, rapid extension is usual
Staphylococcal Scalded Skin Syndrome (SSSS)
The brightly erythematous skin acquire a wrinkled
appearance due to the formation of ill-defined flaccid
bullae filled with clear fluid
Areas of epidermis peel away in response to gentle
stroking (Nikolsky sign) leaving a moist, glistening
denuded area
Crusting around eyes, mouth, and nose produce a
characteristic sunburst radial pattern
Staphylococcal Scalded Skin Syndrome (SSSS)
An acute, sometimes, recurrent, inflammatory
disease of the skin and mucous membranes.
Occur at any age, but more common during childhood
and more frequent in males than in females
Pathogenesis unknown but regarded as a
hypersensitivity reaction triggered by drugs, infections,
and exposure to toxic substances
Erythema Multiforme
Lesions begin as macules or wheals and evolve into
papules or plaques. The center of the lesion may be
vesicular, purpuric or necrotic
Iris or target lesions pathognomonic sign
Skin lesions regress within 2-4 weeks, and heal with
hypopigmentation or hyperpigmentation but without
scarring
Erythema Multiforme
Cutaneous and mucosal lesions have an abrupt onset
following a prodromal respiratory illness
Bullae involved the lips, the mouth, and conjunctiva.
a purulent conjunctivitis is usual as is uveitis
Cutaneous lesions rupture and the denuded skin
may result in significant fluid loss
Erythema Multifome Major (Stevens-Johnson Syndrome)
passage of insufficient volume to maintain
homeostasis usually taken to be:
o <0.5 ml/kg/hr in children
Oliguria
complete cessation of urine
Anuria
normal or excessive urinary output >2
ml/kg/hr in the presence of acutely rising BUN or
creatinine
Polyuria
Defined as the sudden loss of renal function due to inadequate renal perfusion: o Decreased effective circulation o Arterial or venous obstruction o Renal cell injury o Obstruction to urine flow
Acute Renal Failure/ AKI
Reduction in GFR
? Abrupt increase in the blood concentration of creatinine
and nitrogenous products
? Inability of the kidney to appropriately regulate fluid and
electrolytes
Cardinal Feature of Acute Renal Failure/ AKI
Prerenal
? Renal
? Postrenal
Classification of ARF/AKI
Contribute to 55-60% of ARF
Prerenal failure
Also called prerenal azotemia
Prerenal ARF
Transient diminished effective circulating arterial volume
? inadequate renal perfusion ? decrease in the delivery
of oxygen and energy sources ? decreased GFR
Prerenal ARF
Complete recovery of renal function expected upon
return of renal perfusion
Prerenal ARF
Decreased effective intravascular volume
o GI losses (vomiting, diarrhea, nasogastric drainage)
o Renal losses (salt-wasting nephropathy, diabetes
insipidus, drug-induced or osmotic diuresis)
o Hemorrhage (surgery, trauma, GI bleeding)
o Insensible losses (burns, hyperthermia)
o Redistribution (sepsis, heart failure, liver failure,
third space losses, hypoalbuminemia)
Prerenal ARF
constriction due to RAAS activation
Prerenal ARF
Contributes to 35-40% of ARF
Renal ARF
Includes a variety of disorders characterized by renal
parenchymal damage, including sustained
hypoperfusion/ischemia
Renal ARF
Causes: o Glomerular Glomerulonephritis ? Post-infectious ? Lupus ? HSP ? Membranoproliferative (?BP, hematuria, persistently hypocomplementemic) ? Anti-GBM
Renal ARF
Vascular
? HUS (triad: thrombocytopenia, microangiopathic
hemolytic anemia, uremia; with prior hx of
diarrhea d/t Shiga toxins ? endothelial injury
? RF)
? Malignant hypertension
? Renal artery / vein thrombosis
Renal ARF
o Interstitial disease
? Acute interstitial nephritis (infectious, allergic,
drug-induced)
Renal ARF
Tubular disease ? Acute tubular necrosis o Ischemic hypoxic insults o Nephrotoxins o Drugs (i.e. aminoglycosides) ? Intratubular obstruction o Pigment nephropathy (i.e. in hazing, the muscles release myoglobin pigments ? deposition in kidneys) o Tumor lysis syndrome (lysed cells ? release of nucleic acids, crystals, etc. ? deposition)
Renal ARF
? Contributes to 5% of ARF
Postrenal ARF
Includes a variety of disorders characterized by
obstruction of the urinary tract distal to the kidney
Postrenal ARF
? Causes: o Posterior urethral valves (urinary stream is weak instead of parabolic) o Ureteropelvic junction obstruction o Ureterovesical junction obstruction o Ureterocele o Tumor o Urolithiasis (the stone/s should be lodged either in both ureters or in the bladder neck to cause postrenal ARF) o Hemorrhagic cystitis o Neurogenic bladder
Postrenal ARF
? Oliguria ? Edema ? Hypertension ? Dyspnea ? Pallor ? Vomiting ? Lethargy ? Anorexia
Clinical Manifestations of ARF
Decrease in GFR ? decrease in excretion
? Metabolic acidosis
? Common cause of death in ARF!
Hyperkalemia
Schwartz
formula
k x L / PCr
Most important factors in determining cause of ARF
Urinary sediments
most commonly employed in
neonates and infants with ARF
peritoneal dialysis
useful in patients with
relatively stable hemodynamic status
intermittent hemodialysis
useful in
patients with unstable hemodynamic status,
concomitant sepsis, or multiorgan failure in the
intensive care setting
continuous renal replacement therapy
Appropriate use of diuretics
? Normalize blood pressure
? Hydrate and preserve intravascular volume to maintain
adequate renal perfusion
? Avoid potentially nephrotoxic substances in patients with
impaired kidney function (NSAIDs, aminoglycosides,
radiographic contrast agents, general anesthetics, ACE
inhibitors, amphotericin B, and chemotherapeutic drugs)
Prevention of ARF
Defined as either renal injury (proteinuria) and/or a GFR
3 months
? May be the result of congenital, acquired, inherited, or
metabolic renal disease
Chronic Kidney Disease
Congenital abnormalities:
? Dysplasia
? Obstructive uropathy
? Renal hypoplasia
AGE
Less than 5 years old
Acquired diseases (various forms of glomerulonephritis)
Inherited disorders (familial
juvenile nephronophthisis,
Alport syndrome)
More than 5 years old
Kidney damage with
normal or increased GFR
GRF more than 90
Stage 1
Kidney damage with
mild decrease in GFR
GFR 60-89
Stage 2
How much weight does a newborn lose in first week?
When does she regain it?
10%
Regains by 3rd week
Birthweight doubles by…
Birthweight triples by…
5 months
1 year
A couple of first time parents come in with their 2 week old concerned that she weighs less than her birthweight. What do you do?
Reassure. It is okay to not gain weight within first 3 weeks of life.
What is the average birth length?
50 cm
Birth length goes up by 50% by what age?
It doubles by what age?
It triples by what age?
1 year
4 years
13 years
What is the normal head circumference for a term newborn?
35 cm
What is the growth rate for head circumference?
1 cm/month for 6 months and 0.5cm/month from 6 months to 1 year
Define Macrocephaly
Born with normal size head but progress to 98th percentile by six months. This is normal. Kids have normal development.
A 6 month is brought to you for a well visit. You note that the head circumference has gone from 50th percentile to 98th percentile. Development is normal. What do you do?
No workup. Measure parents heads.
A 2 month old presents with a large anterior fontanelle. His development is normal and has been feeding well. Physical exam is otherwise normal. What is your diagnosis? Next steps?
Macrocephaly. No further workup, except measure parents’ heads.
What is Microcephaly?
Head Circumference > 3 SD below the mean for age and gender.
What is Acquired Microcephaly
Microcephaly presenting in child with normal presentation at birth.
A 3 month old is brought in for well visit. Your medical student notes that the head circumference has slipped down the growth curve while the weight and height remain normal. He would like to discharge the patient, is this okay?
No. Acquired Microcephaly needs a workup.
A child is brought to you in the ED because she has not been growing. The mom says she is giving her appropriate formula. What do you check next?
Observe mother/child interaction and feeding technique…AS INPATIENT
What are likely etiology in each case?
- Decreased weight then length
- Normal weight but short
- Microcephalic and dysmorphic
- Inadequate Caloric intake
- Endocrine problem
- Genetic chromosomal problem
5yo girl comes in for well visit. The nurse notes that the child is at normal height and weight but when you look at the curve you note she has only grown 3 cm over the last year. What do you do?
Evaluate for hypothyroidism and growth hormone deficiency because she has grown less than 5cm in one year.
You are presented with a 4yo who was at 50th percentile for ht and wt at birth but is now 25th for weight and below 5th for height. No family history of short stature. What is cause of short stature?
Growth Hormone Deficiency
What is catchy phrase” for constitutional growth delay?”
Parents have short stature
What is the catchy phrase” for inadequate nutrition in short stature patient?”
Weight is off the curve or not within normal range.
True or False
One must have either limitations in intellectual functioning or limitation in adaptive behaviour before age 18 to be called mentally retarded.
False. They must have limitations in both intellectual funtioning and adaptive behavior
What are the classifications of mental retardation and corresponding IQs.
Mild 50-69
Moderate 35-49
Severe 20-34
Profound <20
What is the best guage of cognitive function?
a. language
b. motor
c. social
d. fine motor
a. language
Child must visually fixate by what age?
2 months
Child must visually track and have steady head control by what age?
4 months
Child must turn to sound or voice by what age?
6 months
What age should you be worried if a child does not babble and can not sit?
9 months
At what age should you worry if a child can not walk independently?
18 months
At what age should you worry if a child does not use single words?
24 months
At what age would you worry if patient can not speak 3 word sentences?
36 months
At what age would you worry if a patient has unitelligible speech?
Older than 36 months
How do you diagnose autism?
Patient must present with one of the below before age 36 months
Serious social impairment
Delayed language development
Compulsive behaviours
What test must you do before diagnosing autism?
Hearing test
What one single criteria will rule out autism from the differential in pervasive development disorder.
Normal social interaction.
What distinguishes asperger’s syndrome from the other pervasive development disorders?
Patient’s have no speech or language or cognitive dysfunction. They ONLY have problems with social interaction.
What distinguishes Rett’s syndrome from the other pervasive development disorders?
The child is normal for first year, then regresses.
True or False:
Social difficulty is considered a Learning Disorder.
False. It can be a manifestation of a learning disorder but not a disorder itself.
True or False: Learning disorders must present by the time the child is in elementary school.
False. They often don’t present until the child is older, as kids can compensate in the early years.
True or False: A child can grow out of a learning disorder.
False.
You are seeing a 6yo patient for your collegue. The parents are teachers and are concerned that their daughter is dyslexic because she often mistakes her b and d when writing. What should you tell them?
Reassure them that it is normal for kids under 7 to confuse and reverse the letters b and d.
Define performance IQ and Verbal IQ.
What does it mean when one is significantly different than the other?
Performance IQ measures visual-spatial interpretation. Verbal IQ measures language based learning. A wide gap in the two is a hint that the child is at risk for a learning disorder.
What is the role of achievement testing?
Other than taking your money, they in theory distinguish between potential and actual IQ.
True or False: The predictive validity of IQ testing increases with age.
True.
At what age can a child throw a ball?
At what age can a child throw a ball overhand?
18 mos
24 mos
En ex-30 week preemie is now 8 weeks old. Her mother is concerned because the baby is not able to lift her head from a prone position. What do you do?
Reassure the mother. Preemies are expected to perform and grow at their corrected age. You can stop correcting at age 2yo.
I have a moro reflex, I can look at you but will follow only briefly. How old am I?
Full term newborn
I will look at you, follow you across the room with my eyes and lift my head and shoulders up while resting on my tummy, but can’t really move on my own. How old am I?
2 mos old.
I will laugh and squeal if you entertain me. I will even prop myself up on my arms while on my tummy. I will try to entertain you by rolling from my tummy (prone) to my back (supine), but need help getting back to my tummy. If you hold me up on the table, I can stand and false step. If my hands are free I will reach for your glasses and anything else I can put in my mouth…even though I am not hungry. How old am I?
4 mos. old
I love to interact. I will turn to your voice and if you have anything in your hand, I will surely reach for it and if I get it, I will try to play hard to get and pass it back and forth in my hands. I will even talk in my own language and sit up with a backrest. And if I am lying, I will roll like a roly-poly. How old am I?
6 mos old
I love to sit up and bang blocks together or play pic-a-boo. I will turn to my name and say Mama-Dada but I really don’t know what those words mean. I do know who my family is and get anxious around strangers. How old am I?
9 mos old.
I love going surfing. And when I come back to rest I sit back and say Mama or Dada and immediately my mommy or daddy appears with a cup filled with my favorite drink and feeds it to me, while I hold between my fingers a small sea shell that I picked up on the beach. After I’m done, I help my daddy dress me, but when my sister calls my name and motions for me to go to her, I surf on over!
I am 1 year old. and Life is Good!
Life is not as good anymore. Mom says I have to walk on my own now and I have to drink by myself now. And even worse, today I dropped my sea shell and I had to stoop down, pick it up, stand up and put it in a cup. Since I can still only speak a 3-6 words, I tried writing, but that was only a scribble. I can point to my nose and I do like passing the ball back and forth with my sister. I guess that’s good. How old am I now?
15 months
So I was mouthing off my 10-20 words that I learned, and mom took my hand and walked me up the stairs to my room. I built a tower of two blocks, but I also made a mess, so then I had to vacuum the floor. When she finally brought me down for lunch, she wouldn’t even feed me. I had to feed myself. So I threw a fit and threw the ball. I got in a little trouble, but Mommy is still cool. How old am I?
18 months
My Dad was so proud of me. I built a tower of 4 blocks. I made everyone laugh by dropping a raisin in my milk bottle. How old am I?
16-19 months
I am getting good! I built a tower of 6 blocks today. I was so proud, I jumped up and down and kicked the ball down the steps. Then I took off my clothes and I washed and dried my hands. I got in trouble for that. I can speak about 50 words now and my favorite word is me.” People who don’t know me say they don’t understand half of what I am saying. How old am I?”
24 months
Mommy says I am turning into a chatterbox now, and people can understand almost everything I say. This morning after breakfast I went up the stairs by myself and put on a shirt and shorts for school. In school, I copied a circle and a line with my crayon. Me and my friend Suzy rode our tricycles around the block. Then we stood on one foot to see who could stand longest. We both started laughing cause we could only do it for a couple seconds.
3 years old
Daddy calls me Curious George because I keep asking so many questions. I have to get dressed and brush my teeth. Suzy is coming over again. I think we will play airport today. We both love going up and down the stairs. We are getting better at standing on one foot now. And we can draw a person and know 4 colors. How old are we?
4 years old
I know what silly means. It means I am acting funny. I can count to 5 now and I can draw a person with a head, eyes, nose, mouth, arms, and legs. I am making cereal before I go play board games with Suzy today. How old am I?
Five years old
Hooray I get to ride my bike to school. I need to make sure and tie my shoes so I don’t fall. I have to turn left and then right and then we will be at school. Look, there are ten kids wearing the same uniform as me. That’s why I have to write my name on my sticker. How old am I?
Six years old.
At what age can kids draw the following shapes: Circle Square Triangle Diamond Cross
3 yo 5 yo 6 yo 7 yo 4 yo
A child is brought in for a well visit. The parents are both professors at Hopkins and are very distraught because their 4 year old stutters. Who do you refer them to?
No one. You reassure them. Stuttering is normal until school age.
At what age can a child build a tower of:
2-3 blocks?
4-6 blocks?
8 blocks?
18 mos
24 mos
36 mos
A 3 yo child who is of a bilingual family only has a vocabulary of 20 words. The family feels that this is not a big deal because the child is bilingual. For the boards, is this true?
No. This is not an excuse for delayed speech.
A 2 year old is brought to your office because she is not speaking any clear words. You find out that she had neonatal meningitis and hyperbilirubinemia. What is your next step?
Do a hearing screen.
What are risk factors for delayed or absent language development?
Risk factors are TORCH infections, hyperbilirubinemia, meningitis.
True or False:
Children may be diagnosed with ADHD if the level of inattention and activity is greater than that expected for their MENTAL age as determined by IQ.
True. For example, if a 10-year-old boy has an intelligence quotient of 60, he would be expected to have the attention span and activity level of a 6-year-old.
Which child would MOST benefit from a non-stimulant medication?
A) an inattentive child
B) a hyperactive child
C) a child with both
B
True or False:
A one-to-one aide usually is not necessary for children who have levels of hyperactivity and inattention typical of ADHD.
True. A one to one aide is most beneficial for kids who have destructive behavior or autism or cerebral palsy
Physical examination results are normal for a 2-year-old girl during a health supervision visit. She follows two-step commands, speaks 10 words, points to pictures that you name, and sorts objects by color. She uses a spoon well, but does not use a fork. She plays alongside other children but does not share toys. Her mother would like to know if she should be concerned about the girl’s development.
Of the following, your BEST response is that her daughter
A) has normal development
B) is delayed in her expressive language skills
C) is delayed in her fine motor skills
D) likely has a hearing impairment
E) needs more social interaction with other kids
B. Ten words is about the verbal level of a 16-18 month old.
True or False:
Breast feeding for the first 6 months of life reduces the risk of eczema.
False. It only delays the onset, and the risk beyond age 2 years is unchanged.
True or False:
Breast feeding or hypoallergenic formula reduce the risk of asthma, seasonal allergies and allergic gastroenteritis.
False.
A 4yo is diagnosed with latex allergy. Which fruits should she avoid?
Papaya, avocado, banana, chestnut, passion fruit, fig, melon, mango, kiwi, pineapple, peach and tomato.
A 32yo mother of a 3yo who has multiple food allergies is pregnant again and would like to know if she should try an elimination diet to reduce the risk of her second baby having so many allergies. What do you say?
No. Elimination diets do not reduce the risk of allergies.
A 2yo presents to your office with moderate to severe atopic dermatitis The parents ask you what they can do, which foods could be triggering it. You are referring to an allergist for specific food testing, but in the meantime what should they do?
A) Begin allergy shots
B) Eliminate Eggs, and Cow’s Milk
C) Eliminate Peanuts
D) Eliminate Eggs, Cow’s Milk and Soy Milk
E) Nothing
E.
A 5yo presents to your office as an ED followup for asthma. His mother asks if he is at risk for anything else. How do you respond?
Any child who has one of the three below is at risk for the other two.
Asthma
Eczema
Allergic Rhinitis
What is the risk for eczema if one parent has it? What if both have it?
50% and 70% respectively
How do you confirim allergic rhinitis vs. a simple cold in a 10yo with runny nose, sneezing?
Allergic rhinitis will have eye symptoms (itching, swelling, tearing) and will also have eosinophils in the nose.
True or False:
Non-allerigic rhinitis with eosinophilia syndrome (NARES) presents with eosinophils on nasal smear and positive skin test and elevated serum IgE.
False. The serum IgE would not be elevated.
What is the first step in treating allergic rhinitis?
Eliminate the offending organism
What is the most effective treatment of allergic rhinitis?
Immunotherapy
What are the indications for immunotherapy for allergic rhinitis?
Child over age 5yo and persistent symptoms despite avoidance of allergen and medication.
What is the most effective class of medications for allergic rhinitis?
Nasal steroids
An 8yo child presents after a severe allergy to cat dander. Her mother is a veterinarian, but the family has taken extensive precautions short of mom switching careers. Can the child receive immunotherapy?
YES
What are the catchy phrases for infectious rhinitis?
Young child and winter
What is the defining element of vasomotor rhinitis? What are 5 triggers?
Post-nasal drainage unrelated to specific or infectious agent. Triggers are: 1. Emotions 2. Temperature change 3. Humidity change 4. Cold draft 5. Pollutions
What is rhinitis medicamentosa?
Rebound reaction to adrenergic nose drops.
A 3yo is brought to your office. Her mother is concerned because the child has relentless runny nose. The child’s older sister has severe spring allergies. The medical student would like to start allergy shots in her 3yo to help this child. What do you suggest?
The child does not have pollen allergy because it requires years of exposure. Besides she is too young for allergy shots. She likely goes to preschool and has recurrent URI
What is the primary trigger of anaphylaxis in children? A) Bee stings B) Pollen C) Medication D) Food allergy E) Pet dander
D.
True or False:
Sunflower oil and Hot Pressed peanut oil need NOT be strictly avoided by those with peanut allergy.
True. Sunflower seeds are often processed with peanuts, so avoid those. And cold pressed peanut oil is also a problem, but well refined, hot pressed peanut oil is okay.
What are the most common food culprits in
Toddlers?
Older kids?
Toddlers: Eggs, Milk, Wheat and Soy
Older Kids: Shellfish and peanuts
You receive a call from the school nurse who is seeing a 6yo just after lunch. The lunch was from home and prepared by her dad. The child is not feeling well and has a stomach ache but no respiratory distress or rash. The child has a known milk allergy. The nurse gave mylanta but wants to know what else she should do. What do you suggest? A) Send the child back to class B) Give the child zantac C) Give the child benadryl D) Give Epi-Pen E) Call EMS
D. Abdominal pain and general discomfort could be the only signs of an anaphylactic reaction. Antihistamines may provide symptomatic relief but will never be the right answer in treatment of anaphylaxis
You receive a call from the school nurse who is seeing a 6yo just after lunch. The lunch was chili from the cafetaria. The child has a stomach ache but no respiratory distress or rash. The nurse gave mylanta but wants to know what else she should do. What do you suggest? A) Send the child back to class B) Give the child zantac C) Give the child benadryl D) Give Epi-Pen E) Call EMS
A. This child has food sensitivity likely due to either spicy foods or beans.
In addition to allergic reaction what else presents with acute eosinophilia?
Parasitic diseases
What is the definition of chronic urticaria?
urticaria that lasts longer than 6 weeks
How do you treat chronic urticaria?
Fexofenadine
How does treatment with benadryl and prednisone prevent contrast media reaction?
Contrast media reactions are osmolality-hypertonicity reactions that trigger degranulation of mast cells and basophils with the release of mediators that then cause the reaction. Benadryl and Prednisone inhibit the response caused by the release of these mediators.
A 5yo boy has had previous severe allergic response to shrimp. He now comes in with abdominal pain and needs a CT with IV and PO contrast. What do you do?
A) Go ahead with the CT with IV and PO contrast
B) Go ahead with the CT with PO contrast only
C) Go ahead with the CT with IV contrast only
D) Go ahead with the CT with IV and PO contrast but pre-medicate with benadryl and prednisone
E) Avoid the CT
A. There is no association between shellfish allergy and contrast reaction.
What is the most effective preventive treatment for a child who has had a life threatening bee sting anaphylactic reaction.
Venom immunotherapy is 98% effective in prevention of future reaction.
What are 4 findings typical of ragweed reaction?
Sneezing, Wheezing, Squeezing (chest tightness), plus teary eyes
What is a type I allergic reaction?
IgE mediated anaphylaxis
What is a type 2 reaction?
Mediated by antibodies
What is a type 3 reaction?
Immune complex reaction
What is a type 4 reaction?
Delayed hypersensitivity
True or False:
Antidepressants can interfere with allergy skin testing.
True. They sometimes contain antihistimines which interfere with allergy testing
True or False:
Trauma during testing may give a false positive skin test.
TRUE
A 4yo visits his pediatrician and is prescribed amoxicillin for an ear infection. Two days after beginning amoxicillin, he develops a rash all over his body. His mother brings him to the ED and is concerned that this is an allergic reaction to penicillin. How do you proceed.
A)Stop amoxicillin
B)Continue amoxicillin
C)Consult an allergist
D)Hold amoxicillin for a day and see if the rash clears.
B. If this were an actual allergy to amoxicillin, it would have occured within 1 hour (at most 24 hours) of giving the medication. True allergies would be IgE mediated. This child likely has a viral exanthem or a non-IgE mediated reaction.
Which is the one antibiotic that can be skin IgE tested?
Pennicillin
What defines a positive skin test?
Local reaction within 24 hours of administering the allergen. It represents IgE response. If not within 24 hours it is not an IgE response.
E) Continue formula and workup for an infection.”
A. This is a real milk allergy. This child should be closely monitored and pediatric allergist should be consulted. If it were an eczematic rash, that would be t-cell mediated milk intolerance which could be managed by switching formulas
A 4yo is brought in with itchy eyes and runny nose that began when the family started doing spring cleaning. The grandmother feels that the allergies are likely due to the new cat in the house. She would like to remove the cat. Do you agree?
No. Kids have an emotional attachment to pets, so while removing the allergen is usually the right answer, here she could be allergic to dust from the cleaning and before removing the pet she should be allergy tested.
What are 3 advantages of RAST testing over Skin testing?
- Rast testing is in vitro, so there is no risk of anaphylaxis
- RAST can be done even if there is not enough exposed skin to do skin testing as in severe eczema
- RAST is not impacted by antihistamine use
What are 2 advantages of Skin testing over Rast testing?
- It is more sensitive than RAST testing
2. It is less expensive than RAST testing
A father comes in with his 4yo daughter frustrated because she seems to either catch a cold or diarrhea every month. What do you do?
Reassure him that normal children may have one infection per month especially when in preschool or daycare.
A 4yo girl is brought in by her father as an ED followup because she seems to have recurrent urinary tract infections almost one every month. She is on the 10th percentile for height and weight and her parents are of average height. What do you do?
Pursue further investigation such as a VCUG and perhaps refer to an immunologist.
A child has recurrent pyogenic infections. What is this associated with?
Burton’s B-Cell dysfunction
A child has chronic or recurrent candida infections of the nails, scalp or mouth. What is this associated with?
T cell dysfunction
A child has thrombocytopenia with petichiae and eczema? What do you suspect?
Wiskott-Aldrich syndrome
What is the underlying problem in Severe Combined Immunodeficiency?
Absence of lymphoid tissue
What are the x-ray findings of DiGeorge syndrome?
absence of thymic shadow
What is the key component in adaptive immunity?
Antigen presenting cells
What would be the expected finding on CBC with diff in a patient with T-Cell dysfunction?
Low lymphocyte count because the majority of lymphocytes are T-cells
A 4yo child presents to your office with a wobbly gait and recurrent sinus infections. You note discoloration of her conjunctiva on exam. What is your diagnosis?
Ataxia Telangectasia
True or False:
The T-cell count is often low in Bruton’s Disease.
False. The T cell count is often elevated in Bruton’s.
What is the other name for Bruton’s disease?
X-linked agammaglobinemia
A 9month old is brought to the office with his 3rd ear infection. His mother is a pediatrician and is concerned because most kids don’t get ear infections this early in life. What would be the best approach to coming up with a diagnosis?
Measure immunoglobulins. When they are all low, measure B and T cells.
What is the treatment for Bruton’s disease?
IVIG
What are two high risk severe complications of Bruton’s disease?
Chronic pulmonary insufficiency and bronchiectasis
What is the most common primary immunodeficiency?
CVID. B cells do not become plasma cells, so no immunoglobulins and there is a T cell problem as well.
What are theo most common infections in kids with Common Variable Immunodeficiency?
URI
Lower respiratory infection
Recurrent herpes and zoster
A an 8yo with hypothyroid disease and JRA comes in with sore throat, malaise and weight loss and diagnosed with mononucleosis
What is her most likely underlying diagnosis? What is she at risk of developing?
CVID.
She is at increased risk of developing EBV related lymphoma
Why do you give kids with common variable immunodeficiency IVIG?
To prevent from bacterial infections.
True or False:
Most complement disorders are x-linked.
False. Only properdin deficiency is the only X-linked disorder. Most are autosomal recessive.
A 14yo girl has had 3 episodes for meningicocci, what is highest on your differential for underlying problems?
Complement deficiency
Describe DiGeorge’s syndrome
CATCH 22
Deletions are on the long arm of chromosome 22.
Cardiac defects, Abnormal face, Thymic hypoplasia, Cleft Palate and Hypocalcemia
A neonate has a dysmorphic face, a holosystolic murmer at the left sternal border, and presents with diarrhea and tetany. What do you see on x-ray? What would the calcium level be?
Absent thymus. low calcium. no parathyroid. This is DiGeorge syndrome.
What is the treatment of DiGeorge syndrome?
What is the general prognosis?
Thymic Transplant. Poor. They often die from sepsis.
What is X-linked Hyper IgM Syndrome?
Man who has gone hyper after losing his CD40, causing disruption of this boy.
CD40 ligand is missing, so there is disruption of B-cell differentiation.
How do kids present with X-linked hyper IgM syndrome?
Boys age 6mos - 1 year who have frequent OM and Sinupulmonary infections and diarrhea. Labs show low IgA, IgE, IgG but high IgM
An 8 month old who was not born to an HIV mother or father, is diagnosed with PCP pneumonia. What does this child have?
Hyper IgM syndrome where a T cell abnormality prevents conversion of IgM to IgG.
For which immunodefficiency is Ig replacement most appropriate?
x-linked hyper IgM syndrome.
True or False:
Over 80% of patients with Selective IgA deficiency are asymptomatic.
TRUE
What is Job Syndrome?
Hyper IgE syndrome.
What are the three major characteristics of hyper IgE syndrome?
Eczema
Eosinophilia
E on the nose (sinopulmonary infections)
Aside from Eosinophilia, Eczema and Sinopulmonary infections. What are 3 other frequent complications of Job’s Syndrome?
Kids with Job Syndrome tend to get infections with Staph aureus and chronic thrush as well as fractures and other skeletal abnormalities.
What differentiates Job Syndrome from atopic dermatitis?
Eosinophilia, Skeletal problems and abnormal facies.
What differentiates Job’s Syndrome from Wiskott Aldrich?
Wiskott Aldrich has bleeding from the thrombocytopenia
How do you treat Job’s Syndrome?
Antibiotcs and steroids
What is SCID?
Severe Combined Immune Deficiency caused by abnormal B and T cells
What are the typical presentations of kids with SCID?
- Present before age 3 months
- Otitis
- Thrush
- Dermatitis
- Diarrhea
- FTT
True or False:
SCID may have normal B cell count
TRUE
What is the diagnostic test for SCID?
Flurometric analysis of B, T, and NK cells. There will be low to no T cells.
What enzyme deficiency did the Bubble Boy have when he appeared on Seinfeld?
ADA deficiency leading to B and T cell dysfunction (SCID)
What is transient hypogammaglobinemia of infancy?
Transient decrease in IgG and IgA which presents around 6 months of age when mom’s IgG is breaking down. They usually outgrow it.
What is the hallmark of transient hypogammaglobinemia of infancy?
Very low IgG in a 6-7 month old
What is the hallmark of Wiskott Aldrich?
What is the immune deficiency?
What is the cure?
Thrombocytopenia and low IgM
Bone Marrow Transplant
What is chronic granulomatous disease?
It is a phagocyte dysfunction in which phagocytes are not able to kill ingested bacteria and fungi.
What is the genetic transmission of Chronic granulomatous disease?
2/3 are x-linked and rest are autosomal recessive
Which organs are most affected in Chronic granulomatous disease?
The front line organs. Skin, GI, Spleen, Lymph, Lungs and Liver.
How do most kids with Chronic granulomatous disease present?
Before age 5 and with multiple Staph aureus abscesses or recurrent UTI with Serratia
What is the diagnostic test for Chronic granulomatous disease?
Nitrobluetetrazolium (NBT)
What is the treatment for Chronic granulomatous disease?
Aggressive antibiotics and interferon gamma
A neonate has not had separation of her umbilical cord and it is now about 4 weeks. What do you suspect?
Leukocyte adhesion deficiency.
A 4yo has recurrent perirectal abscesses. The mother is hyper about hygiene but these boils don’t seem to heal despite antibiotics. What is your suspicion?
Leucocyte adhesion deficiency
What is the cure for leukocyte adhesion deficiency?
Bone Marrow Transplant
True or False:
A child who has acquired HIV from his mother may have elevated immunoglobins during his first year of life.
TRUE
What is the mechanism of action of the following medications?
Zidovudine
Nevirapine
Indinavir
Zidovudine is a Nucleoside analog
Neveripine is a reverse transcriptase inhibitor
Indinavir is a protease inhibitor.
True or False:
HIV treatment usually involves 2 protease inhibitors, and 2 nucleaoside analog.
False it is 2 nucleoside analogs and one protease inhibitor
What does NBT test?
Neutrophil activity (not number). Normal is blue. Clear is a sign of Chronic granulomatous disease
What does CH50 test?
When do you order it?
Complement disorder. Order when a patient has repeated serious bacterial infections.
What is the TB/Candida test useful for besides TB?
Any t-cell disorder such as AIDS
A child is healthy until age 6 months but then begins to recurrent ear infections and diarrhea. What is your test of choice? A) CH50 B) NBT C) Measure immunoglobulins D) TB Skin Test E) Rebuck Skin Window
C. This child had normal humoral system because she was relying on mom. Now mom’s system has faded, so she’s got a be a stand up comic on her own.
What does the Rebuck skin test measure?
The ability of cells to migrate. It is useful in diagnosing leukocyte adhesion deficiency.
