amboss Flashcards
what are characteristic mulluscum contagiosum
nontender, skin-colored, pearly, dome-shaped papules with central umbilication. They are usually 2–5 mm in diameter and commonly occur on the trunk, face, and genitalia.
what is the treatment for mulluscum
nothing. usually revolves within 6-9 months
measles presents how
cough coryza koplik spots.
what are the stages of chickenpox
papules, vesicles, crusts
remember severe pruritus.
what is the course of the measles rash
face, downward and outward
what is the typical course of measles
10-12 self-limited. can cause severe pneumonia, acute otitis media, diarrhea, encephalitis
what is the typical presentation of roseola infantum
characterized by a 3-day high fever (causing a tonic-clonic febrile seizure in this child) that ends abruptly (“3-day fever”) and is immediately followed by a blanching, maculopapular, nonpruritic rash. The rash appears mainly on the trunk but may spread to the face and extremities. It is commonly associated with cervical, postauricular, and/or occipital lymphadenopathy.
what is roseola caused by
herpes virus 6
/what is the first line treatment for ectopic dermatitis or eczema
topical emollients
what is used to treat mild impetigo
mupirocin
what is muprocin
this is an antibiotic ointment used for the
what is the course of action if a pregnant mother has a child that is sick with parvovirus B19/fifths disease
check the mothers immune status. If she is immune with IgG then nothing is required.
Is someone with fifths contagious after the rash has appeared
no.
what is the course of action for a mother with confirmed parvovirus infection who is pregnant
monitor with serial ultrasounds
Bullous impetigo is
a disease caused by the exfoliative toxin A from Staphylococcus aureus and is characterized by a pruritic rash that predominantly affects the trunk. The rash is typically comprised of flaccid and superficial bullae, which may rupture and leave thin brown crusts. Lateral traction causes sloughing of the skin, which indicates a positive Nikolsky sign.
Bullous impetigo is treated with
first-generation cephalosporins such as cephalexin.
eczema herpeticum is
symptoms (fever, malaise, lymphadenopathy) cutaneous manifestation of herpes infection (usually HSV-1 or HSV-2). This condition is associated with pre-existing skin conditions, most often atopic dermatitis.
is eczema herpeticum serious
this is a medical emergency as it can spread rapidly
what is the treatment for eczema herpeticum
IV acyclovir
Tinea corporis is
a skin infection caused by dermatophytes of the Trichophyton (most common), Microsporum, and Epidermophyton genera.
what is the treatment for tinea corporis
topical anti fungal such as miconazole
what is the treatment of choice for tinea capitis
oral griseofulvin
what is the normal treatment for cat scratch disease in an immunocompentant
nothing. it is self limiting
what is the treatment for someone with cat scratch disease that has bulky lymphadenopathy and
azithromycin
shown to reduce lymphadenopathy and length of disease
what is the presentation of measles
cough, coryza, conjunctivitis and an erythematous maculopapular, blanching, and partially confluent exanthem on his entire body, generalized lymphadenopathy, and high-grade fever,
what is the test of choice for measles
IgM for measles
what is the treatment for strep throat
oral penicillin.
what is the alternative therapy to penicillin for strep throat
azithromycin
what is the rare complication of measles virus
subacute sclerosing pan encephalitis
what treatment should be administered to all children with measles
vitamin A
why is vitamin A given to children with measles
because it reduces the risk of complications
what are the complications of measles
otitis media, viral pneumonia, encephalitis, or corneal ulceration
Estimated maintenance requirements follow what rule and what is it
4/2/1 rule: 4 cc/kg/hr for the first 10 kg, 2 cc/kg/hr for the second 10 kg, and 1 cc/kg/hr for every kg above 20. Based on patient’s weight, using the same 4/2/1 rule as used to calculate preoperative maintenance requirements.
what fluid should be used to maintain a burn victim
dextrose with LR
what is the presentation of chicken pox
This patient’s constitutional symptoms (fever, headache, and myalgia) along with a highly pruritic, generalized rash with multiple lesions in different stages (macules, papules, vesicles, and crusted lesions) is characteristic of chickenpox.
what is herpangina
Herpangina is most common in children between the ages of 3–10 years and infections occur most frequently in summer and fall. As in this case, the first manifestations of the disease are usually oropharyngeal lesions and associated sore throat. Coxsackie A virus is the most common underlying pathogen. presents as fever, malaise, chills, sore throat and oropharyngeal maculovesicular lesions
what is the rash caused by carbamazepine
this is DRESS drug reaction with eosinophilia and systemic symptoms
presents as a generalized pruritic rash
t with fever, and a diffuse morbilliform rash that progresses to a confluent erythema with accentuation of the hair follicles, as well as facial edema, and lymphadenopathy.
can also present with pneumonitis, nephritis and hepatitis
what is hereditary hemorrhagic telangiectasia
autosomal dominant recurrent episodes of epistaxis and telangiectasias of the lips, nose, and fingers, symptoms that are classic for hereditary hemorrhagic telangiectasia (also known as Osler-Weber-Rendu syndrome). Patients with this syndrome may also present with pulmonary or hepatic arteriovenous shunts, which may lead to high-output cardiac failure.
what is cutis marmorata
bluish mottled skin appearance in the newborn
what is erythema toxicum and where does it appear on the newborn/spared on the newborn
Erythema toxicum appears within the first week of life as small, The rash appears on the trunk and proximal extremities, but the palms and soles are typically spared.
what is the progression of erythema toxicum
red macules and papules that progress to pustules with surrounding erythema.
what is the cause of E tox
It is most likely caused by immaturity of the sweat glands and follicles.
what is the treatment for e tox
the condition is benign and requires no treatment. Complete resolution of the rash occurs within 7–14 days.
what is an absolute necessity before beginning isoretinoid therapy on a female
a pregnancy test
If the initial pregnancy test rules out pregnancy, a primary (e.g., IUD, birth control pill) and secondary (e.g., condoms, cervical cap, diaphragm) contraceptive method must be used starting one month before isotretinoin therapy and continuing until one month after isotretinoin therapy is completed. The patient should also undergo liver function and lipid profile tests (both before and during therapy) since the use of a retinoid is associated with decreased liver function and hyperlipidemia.
why are pregnancy tests and birth control a necessity before isoretinoids in females
because they are high teratogenic
what is the first line therapy for mild noninflammatory acne
topical benzoyl peroxide
Ichthyosis vulgaris is
a common condition (affecting ∼ 1% of the population) that is caused by an autosomal dominant filaggrin mutation, which causes the stratum corneum to dry out. This results in the formation of fine white scales on skin within the first few months of life. Areas of skin that are more moist or less exposed to air, such as the groin, axilla, and cubital and popliteal flexures, are usually spared. During summer or periods of increased humidity, the lesions may disappear as a result of increased hydration of skin by sweat.
what is the most significant modifiable risk factors for SIDS
sleeping the prone position is highly associated with SIDS. thus sleeping in supine is recommended.
Is smoking associated with SIDS
yes. but not the most important risk factor
Should pregnant women be vaccinated for tetanus
yes. protects the baby as well
how does neonatal tetanus present
a life-threatening condition characterized by muscle spasms, myoclonus, irritability, and poor feeding.
what is the presentation of neonatal listeriosis
The presence of severe systemic disease (e.g., fever, hypotonia, respiratory distress) in this infant suggests early-onset sepsis. The presence of seizures is concerning for meningitis. This patient’s multiple visceral granulomas (granulomatosis infantiseptica) is specific for neonatal listeriosis.
what is the best way to avoid neonatal listeriosis
avoiding unpasteurized milk products.
Why cant topical erythromycin be used to treat chlamydial conjunctivitis
because this indicates systemic infection which will lead to chlamydial pneumonia
what is the presentation and treatment for chlamydial conjunctivitis.
watery or mucopurulent discharge in a neonate with proper postnatal care. conjunctivitis. treat with oral erythromycin
what is the presentation of severe airway compromise requiring intubation in the newborn
wheezing, cyanosis, poor respiratory effort
what is the empiric treatment for neonatal sepsis
ampicillin and gentamicin
when does neonatal gonoccocal infection present
usually 2-7 days after birth
Can genital herpes transmit to the baby during childbirth
yes, but usually the infection has to be active.
what is the presentation of neonatal herpes
there is usually a vesicular rash and sepsis
what does blood gush of fluid indicate
premature rupture of membranes
ABO hemolytic disease of the newborn is
a common cause of hemolytic disease of the newborn and can occur even during the first pregnancy. Anti-A and anti-B antibodies are formed early in life, without prior sensitization. IgG antibodies that can enter into fetal circulation during pregnancy and may result in hemolysis when the infant has A or B antigens
The clinical presentation of ABO incompatibility is
typically mild, with hyperbilirubinemia developing within the first 24 hours after birth.
what are the most likely heart defects present in congenital rubella
patent ductus arterioles and pulmonary hypertension
what is the diagnosis and treatment for a baby that feeds for a long time, has failure to thrive and diaphoresis during feeds with a murmur in the pulmonary region (upper left sternal border)
PDA. give indomethacin
are NSAIDs effective in treating full term infants
NO. only preterm infants.
what is neuhauser sign and what does it indicate
A radiographic finding of a mottled (“soap bubble”) appearance in the distal ileum and/or caecum as a result of meconium mixing with swallowed air, often caused by meconium ileus. It can also been seen with other conditions such as necrotizing enterocolitis or ileal atresia.
what is the presentation of meconium ileus
this is failure to pass stool after birth, dilated small bowel with microcolon, infant discomfort
what is the presentation of fetal hydantoin syndrome and what is the cause
phenytoin treatment during pregnancy
e intrauterine growth restriction, craniofacial abnormalities (e.g., cleft palate, hypertelorism, slanted palpebral fissures, and low-set ears), a short neck, phalanx/fingernail hypoplasia, and excessive hair on the body and face.
don’t get fooled by turner’s syndrome red herring
what is the treatment for fetal hydantoin sydnrome
Treatment is largely supportive and includes surgery to fix the cleft palate in addition to special education.
Phenylketonuria is associated with
developmental delay after the age of 4–6 months, blue eyes, pale hair and skin (as tyrosine is a precursor in melanin production), a musty odor, and seizures in ∼ 50% of patients.
Management of phenylketonuria involves
dietary restriction of phenylalanine.
what heart valve condition is assocaited with fragile X syndrome
mitral valve prolapse
what are the characteristics of cephalohematoma
occurs in 1–2% of live births and is palpable as a soft mass that develops slowly over several hours after birth. Being a subperiosteal hematoma, a cephalohematoma is limited by suture lines to the surface of one cranial bone. Forceps delivery and vacuum-assisted vaginal delivery (ventouse) are risk factors. Cephalohematomas usually require no treatment because the hematoma is resorbed within a few weeks.
Subgaleal hemorrhage presents as
a fluctuant scalp swelling and is associated with vacuum-assisted delivery. Because the bleeding is not limited by suture lines, subgaleal hemorrhage is located superficial to the periosteum and is usually associated with features of severe blood loss (e.g., tachycardia, hypotension, pallor).
which vacuum-assisted delivery complication crosses suture lines
Subgaleal hemorrhage
Caput succedaneum presents as
a scalp swelling and is associated with vacuum-assisted delivery. However, a caput succedaneum is usually present at birth, is not limited by suture lines (because it is located superficial to the periosteum), and presents typically as a diffuse, boggy swelling that may shift with movement.
which vacuum-assisted delivery complication is under the periosteum
cephalohematoma
Edwards syndrome (trisomy 18) is characterized by
Prominent occiput, microcephaly, low-set ears, clenched fists with flexion contractures, and convex deformities of the plantar foot (rocker bottom feet) are all characteristic of Edwards syndrome (trisomy 18).congenital cardiac malformations, particularly ventricular septal defects.
when is the booster for pertusis
11-12
what is the in utero presentation of Down syndrome
Increased nuchal translucency, decreased levels of PAPP-A, shortened femur length, shortened fifth digits with clinodactyly, and a small nasal bone in a fetus
what is the characteristic finding suggestive of trisomy 13 patau syndrome
polydactyly
cleft lip and palate
what is required for neonates with a heart rate less than 100
resuscitation with positive pressure ventilation.
what is the presentation of a baby born to a mother that smokes
intrauterine hypoxia presents as polycythemia, hypoglycemia, facial plethora, respiratory distress, cyanosis, apnea, poor feeding, hypoglycemia, and plethora (ruddy complexion
what are some normal findings of female neonates
swelling and erythema of the vulva and vagina with white to bloody mucousy discharge, firm breast buds. this is due to estrogen exposure in utero
Transient tachypnea of the newborn is
a benign condition that usually resolves without treatment within 24-48 hours.
therapy for transient tachypnea of the newborn is
supportive care, including providing supplemental oxygen by hood or nasal cannula (with a goal O2 saturation of > 90 %), maintaining a neutral thermal environment, and providing adequate nutrition. Other causes of respiratory distress should also be excluded (i.e., pneumonia, sepsis, congenital heart condition, RDS).
what is the treatment for a clavicle fracture in the newborn
reassurance and pinning of the sleeve to the chest
what is bronchopulmonary dysplasia
is a chronic lung condition that develops in preterm infants who are exposed to prolonged (>28 days) mechanical ventilation and O2 therapy
presents with respiratory distress sydnrome
what is respiratory distress syndrome
evidenced by his tachypnea, decreased O2 saturation, grunting, moderate subcostal retractions, and hypoxic peripheral vasoconstriction. prematurity causes neonatal respiratory distress syndrome (affects 10% of preterm babies) due to insufficient pulmonary surfactant levels, which causes increased alveolar surface tension and atelectasis (decreased breath sounds bilaterally).
what does a chest X show for neonatal RDS
Chest x-ray showing diffuse, ground-glass densities with atelectasis will confirm the diagnosis.
what is the treatment for neonatal RDS
This newborn would benefit from nasal CPAP to minimize lung atelectasis.
what does gestational diabetes put the fetus at risk for with respect to serum electrolytes
hypocalcemia
hypomagnesemia
what does gestational diabetes put the fetus at risk for with respect to cardiac anamolies
hypertrophic cardiomyopathy
what does gestational diabetes put the fetus at risk for with respect to serum cellular components
polycythemia
n an increased metabolic demand that can lead to fetal hypoxia and increased RBC production to compensate.
For HIV-positive mothers with a viral load of ≤ 1,000 copies/mL, infant HIV PEP
with zidovudine for 6 weeks is effective in preventing neonatal transmission of HIV.
If the viral load is > 1,000 copies/mL (or if the mother is not on antiretroviral therapy during pregnancy), BLANK is recommended for PEP.
a three-drug regimen (such as zidovudine, lamivudine, and nevirapine)
what additional step can be taken to prevent transmission to fetus from mothers with poor HIV contorl
Additionally, delivery via cesarean section would have likely been recommended in this child to further reduce the risk of transmission, although vaginal delivery may be considered in cases of low viral load.
what additional supplement do preterm infants require
iron
Preterm infants have lower iron stores than infants born at term and, due to their rapid growth, require more daily iron than infants born at term. As breast milk does not contain enough iron to meet these requirements, daily iron supplementation is necessary until 6 months of age to prevent the development of iron deficiency anemia.
infants born at home are at higher risk for hemorrhage because
they didn’t receive the vitamin K shot at the hospital
marijuana use during breast is
contraindicated because cannabinoids accumulate in the breast milk. but we dont know what it does.
what is the treatment of choice for a < 1year old with botulism
human derived immune globulin
the equine derived will have a higher chance of reactivity in such a young patient
what is the treatment of choice for botulism in a >1 year old
equine-0derived antitoxin
what is used for postexposure prophylaxis in individuals with an unknown immunization history who have wounds that are neither clean nor minor.
A combination of TIG and Tdap
Orbital cellulitis is most commonly caused by
bacterial spread from ethmoidal or maxillary sinusitis as these sinuses are most proximal to the orbits.
is used for empirical treatment of community-acquired meningitis
A combination of vancomycin and ceftriaxone.
to cover the most likely pathogens in patients between the age of one month and 50 years, including S. pneumoniae, N. meningitidis, and H. influenzae.
what is given for prophylaxis for community acquired meningitis
Rifampin is indicated for PEP in all close contacts (e.g., the patient’s brother and parents).
Treatment of mastoiditis consists of
intravenous antibiotic treatment (e.g., with vancomycin) as well as tympanostomy and subsequent tympanostomy tube insertion in mild cases or mastoidectomy in more severe cases.
what is needed to diagnose mastoiditis
CT scan
what are people with bacterial meningitis at risk for and what are the endocrinological consequences of that
waterhouse-friedichsen sydnrome and adrenal hemorrhage which leads to acute adrenal insufficiency
what is the presentation of Guillain barre syndrome
peripheral neuropathy and ascending paralysis following URI. –absent deep tendon reflexes
protein-cytological dissociation in the CSF
what is the treatment for Guillain barre
IVIG and supportive
empirical treatment for bacterial meningitis in infants younger than 1 month
ampicillin plus an aminoglycoside (e.g., gentamicin) and/or a third-generation cephalosporin (e.g., cefotaxime).
the empirical treatment of community-acquired bacterial meningitis in patients > 50 years of age.
A combination of vancomycin, ampicillin, and cefotaxime is used.
A different antibiotic regimen is used for the empirical treatment of meningitis in neonates.
Ceftriaxone is contraindicated in neonates with hyperbilirubinemia because
it displaces bilirubin from the albumin binding site and is therefore associated with an increased risk of kernicterus.
Trachoma conjunctivitis initially presents
as follicular conjunctivitis and then progresses to a mixed papillary and follicular conjunctivitis, as seen in this case. The patient’s corneal haziness with neovascularization is called pannus and is a classic presentation of active trachoma.
Contraindications for administering pertussis-containing vaccinations include
severe allergic reaction to the vaccine (or to one of its components) and encephalopathy not due to any other cause within 7 days of a prior vaccination. Uncontrolled neurological disorders should prompt a delay of the vaccination until the condition has been sufficiently assessed.
what causes duchennes muscular dystrophy
truncated dystrophin gene –X-linked
strabismus interventions include what
either patching the healthy eye or blurring it with cycloplegic eye drops (penalization), such as cyclopentolate. The increased use of the weaker eye results in the formation of neural pathways and prevents amblyopia.
Can clindamycin or cephazolin cross the BBB
NOPE
what is the empiric treatment for brain abscess
surgery immediately after imaging
what is a child more likely to have a pituitary adenoma or a craniopharyngioma
cranio.
pituitary adenomas are more common in 3-60 age range
what is the most common malignant brain tumor in children
Medulloblastoma
when are Medulloblastomas usually found
with a peak incidence between the ages of 3 and 5.
where are medullobastomas found
The mass is characteristically located in the cerebellum.
symptoms of medulloblastoma
Patients typically present with symptoms of hydrocephalus/increased ICP and involvement of the cerebellar vermis such as vomiting, morning headaches, ataxia, and cranial nerve dysfunction.
Spinal drop metastases are in what percentage of medulloblastomas
found in about 40% of cases.
diagnostic criteria for otitis media with effusion (OME).
A history of recurrent ear infections, language developmental delay, and conductive hearing loss with retracted tympanic membranes that do not move briskly on pneumatic otoscopy
what test is the preferred method for determining the extent of hydrocephalus
ultrasound
what is the treatment for communicating hydrocephalus
VP Shunt
what presentation is most consistent with recurrent complex partial seizures
(focal seizures with impaired awareness), which are caused by abnormal electrical activity arising within the temporal lobe. The muddy taste in her mouth (gustatory aura) marks the onset of the seizure. The ictal phase consists of her staring, non-responsiveness to external stimuli, and automatisms such as facial grimacing and hand gestures. The postictal phase of the complex partial seizure is characterized by lethargy, temporary confusion, and amnesia.
can have young children have migraines and what is the therapy
yes
NSAIDs or acetaminophen are the first line therapy fro abortives in children
what should be done if first line therapy for migraines in children is ineffective
use the other first line therapy. IE if acetaminophen was used then add NSAIDs
type 1 myotonic dystrophy presents as
Abduction of the thumb followed by slow relaxation upon percussion indicates myotonia. Myotonia in a 9-year-old boy with muscle aches and weakness in the distal muscles (hand and/or foot), face, as well as extraocular muscles
Reduced fetal temporalis and pterygoid muscle growth results in a high-arched palate. About 50% of patients with myotonic dystrophy have some degree of cognitive impairment.
what are thee signs indicating Friedreich’s ataxia (FA).
girl presents with progressive bilateral limb ataxia and weakness, loss of deep tendon reflexes, pallhypesthesia, dysarthria, and skeletal deformities, Pes cavus (inverted feet), hammer toes, and kyphoscoliosis are all typical skeletal deformities seen in FA
what is a neurological sequelae of bacterial meningitis
hearing loss
characteristics of Sturge-Weber syndrome.
Most patients with the disorder are born with a port-wine stain (nevus flammeus), as seen in this patient, which usually lies over the region of the trigeminal nerve. The left-sided focal seizures, hypotonia, and absent reflexes are likely due to a right-sided leptomeningeal angioma, which classically occurs on the same side as the port-wine stain. Developmental delay is also common. Abnormal blood vessels of the eye lead to glaucoma, resulting in the cupped optic disc seen in this patient.
A laboratory test in which nonhuman erythrocytes (e.g., from sheep or horses) are exposed to human serum. Aggregation of erythrocytes indicates
the presence of heterophile antibodies (e.g., as a result of infectious mononucleosis).
The physical exam findings are also classic for rickets are
including erosion of tooth enamel, frontal bossing, widened joints, varus knee deformity (genu varum), and beading of the ribs (“rachitic rosary”). this can cause developmental delay and prolonged time to walking
what is leukocytoclastic vasculitis
umbrella term for vasculides such as henoch-scheinlon Purpura
henoch-scheinlon Purpura
HSP is an acute immune-complex mediated vasculitis of the small vessels that is often preceded by an upper respiratory infection. The patient’s history of pharyngitis 2 weeks ago and palpable nonblanching skin lesions with arthritis and abdominal pain are classic signs of HSP. HSP can also manifest with bloody stools or occult bleeding (as in this patient) and is accompanied by a raised platelet count, which helps to differentiate it from thrombocytopenia leading to intestinal bleeding and petechiae. Renal disease is seen in up to 50% of all patients with HSP and manifests with features of nephritic syndrome (e.g., hematuria, proteinuria),
does erythema migrans itch
it can be slightly itchy.
Major criteria for acute rheumatic fever include
arthritis (migratory polyarthritis primarily involving the large joints), carditis (pancarditis, including valvulitis -murmur due top inflammation of the valve), sydenham chorea (CNS involvement), subcutaneous nodules, and erythema marginatum.
Minor criteria for acute rheumatic are
arthralgia, fever, ↑ acute phase reactants (ESR, CRP), and a prolonged PR interval on ECG.
To diagnose rheumatic fever how many major/minor criteria are required
two major or one major plus two minor Jones criteria are required
what is an alternative treatment to penicillin for acute rheumatic fever
clarithromycin
what are the treatments for acute rheumatic fever
first line is penicillin V and NSAIDs + bed rest
alternative is amoxicillin
if allergic to beta-lactams then use clarithropmycin or macrolide
what is the appropriate combined empiric antibiotic therapy for a burn victim with hospital acquired infection
Vancomycin and cefepime for antipseudomonal and MRSA coverage
Sydenham chorea presents how
In a child with a recent history of a sore throat, rapid onset of involuntary purposeless movements, emotional lability, hypotonia, inability to sustain contraction (“milkmaid grip”), and flexion of the wrist and extension of the digits when the arms are extended (“choreic hand”)
what is the cause of pompe disease
A defect in lysosomal acid maltase (also referred to as lysosomal acid alpha-glucosidase)
pompe disease presentation
Without this enzyme, the conversion of glycogen to glucose in the lysosome cannot be completed and glycogen buildup becomes toxic to numerous cells, predominantly skeletal and cardiac muscle cells. Notably, the condition does not present with hypoglycemia or metabolic acidosis because glycolysis and gluconeogenesis do not depend on lysosomal glycogenolysis.
Prenatal alcohol exposure results in fetal alcohol syndrome, which presents how
microcephaly, flat philtrum, short palpebral fissures, hypertelorism, depressed nasal bridge, and micrognathia. behavioral problems (e.g., hyperactivity and inattention) and intellectual disability (e.g., learning disabilities, memory and reasoning deficits, and impaired language development) , ventricular septal defect
von Gierke’s disease (type a) caused by and presents
Glucose 6-phosphatase deficiency
with failure to thrive, lethargy, hepatomegaly, severe fasting hypoglycemia (causing hypoglycemic seizures), hyperlipidemia, and lactic acidosis.
The most common form of galactosemia is which leads to what
galactose-1-phosphate uridylyltransferase deficiency, which leads to the accumulation of galactose-1-phosphate and galactitol. Infantile cataracts are caused by accumulation of galactitol in the eye lens.
when do the symptoms of galactosemia begin
when the baby starts feeding
treatment of galactosemia
avoid lactose
what are infants with galactosemia at increased risk for
sepsis from e coli
adrenoleukodystrophy characteristics include
Rapidly deteriorating vision, hearing, motor abilities, and cognition in the presence of spastic tetraplegia, sensory loss, and adrenal insufficiency (suggested by hyperpigmented skin) in a young boy . A family history of what was likely the same condition in a maternal relative supports the diagnosis of this X-linked recessive condition
cause of adrenoleukodystrophy
peroxisomal ATP-binding cassette transporter dysfunction
cri-du-chat syndrome is characterized by
Failure to thrive, difficulty feeding, a squeaky (i.e.high-pitched) cry, and cardiac anomaly (possibly a ventricular septal defect) in a child with down-slanting palpebral fissures, single palmer crease, round face
cause of cri-du-chat
micro deletion of chromosome 5
is turners syndrome associated with intellectual disability
NO
what is associated with turners
hypertension, wide-spaced nipples, aortic insufficiency because of bicuspid aortic valve and coarctation. low set hairline and lymphedema
Over 98% of men with what have congenital bilateral absence of vas deferens, which results in azoospermia and infertility.
cystic fibrosis
what nasal phenomenon is associated with cystic fibrosis
polyps
what coagulopathy is found in cystic fibrosis and why
Exocrine pancreatic insufficiency arising from hyperviscous secretions in cystic fibrosis leads to malabsorption, steatorrhea, and deficiency of fat-soluble vitamins (A, D, E, and K). Vitamin K deficiency causes coagulopathy and an elevated prothrombin time.
what acid base disorder is found in cystic fibrosis and why
metabolic alkalosis
Patients with CF may lose an excess of chloride in their sweat (due to defective chloride channels) and GI tract, resulting in chloride-dependent metabolic alkalosis, not acidosis.
what pulmonary phenomenon is often found in cystic fibrosis and why
recurrent pulmonary infections and chronic bronchitis in CF lead to obstructive lung disease with increased air trapping and lung hyperinflation, resulting in increased, not decreased, residual volumes.
what is highly associated with cystic fibrosis
meconium ileus
most infants with meconium ileus have cystic fibrosis
what is the presentation of lesch-Nyhan sydnrome
usually asymptomatic for the first few months of life and then present with hypotonia and developmental delay. As the disease progresses, deficient HGPRT leads to neurological symptoms (cognitive impairment, dystonia, spasticity), behavioral symptoms (aggression), and elevated serum uric acid as well as its accumulation in peripheral tissue, which results in gouty arthritis, urate nephropathy, and megaloblastic anemia. A hallmark of the disease is self-injurious behavior including chewing the fingers, biting the lips, and banging of the head or limbs.
homocystinuria causes and presentation
Cystathionine synthase deficiency
Marfanoid habitus, high-arched palate, inferior lens dislocation, kyphosis, hyperelastic skin, hypermobile joints, and possibly intellectual disability (as indicated by delayed language, block-building, and drawing abilities). excessive homocysteine and sodium nitroprusside will turn an affected patient’s urine a deep red color,
easy way of thinking about homocystinuria
marfans with developmental delay
what is the cause of alport syndrome
type IV collagen defect
what is the cause of henoch-schonlein purpura
thought to arise from IgA immune complex deposition in vascular walls, which results in activation of complement factors and organ damage.
risk factors for febrile seizures
high fever, viral infection (especially HHV-6 and influenza), a family history of febrile seizure, and recent immunization (especially DTP and MMR) are all known risk factors.
what is the presentation of herpes conjunctivitis
HSV can cause nonpurulent conjunctivitis, which presents with photophobia, pruritus on the periocular area, and crusts over the eyelashes, as well as conjunctival injection, redness, and watery discharge from the eye on examination. HSV-1 conjunctivitis is more common in children and adults, while HSV-2 often affects neonates. HSV conjunctivitis is usually unilateral and often presents with a vesicular rash.
patients with a history of what are particularly susceptible to HSV conjunctivitis.
atopic dermatitis
what is the next step for a baby showing signs of sepsis without a source from urinalysis, no signs of intracranial pressure and no neurological signs on exam
In infants < 1 month old with fever, poor feeding, jaundice, and irritability or lethargy, what needs to be ruled out!
lumbar puncture
meningitis
what is the most common brain tumor found in children and where is it usually located and how does it present
Pilocytic astrocytoma and is usually located within the posterior fossa. Cerebellar compression can cause falls and dysdiadochokinesia, while increased intracranial pressure can result in lethargy, morning headaches, nausea, and vomiting. Imaging typically shows a well-circumscribed, enhancing cystic mass within one of the cerebellar hemispheres.
what is the prognosis (generally) for pilocytic astrocytoma
Since pilocytic astrocytomas are low-grade tumors (WHO grade I), patients usually have a favorable prognosis following surgical resection.
what are the nucleotide repeats for friedrichs ataxia
GAA in the FXN gene
what is the treatment course for a mild concussion
observation for 6 hours, then discharge. have a responsible individual monitor the patient for 24 hours and return if neurological symptoms occur. refrain from contact for 1 week
what is the presentation and management of congenital torticollis
A conservative approach such as stretching program with congenital torticollis, which typically presents with a tilted head, limited range of motion, and a palpable, well-circumscribed mass within the sternocleidomastoid muscle (SCM) due to unilateral fibrosis.
how can brachial plexus injuries in the newborn present
horners, reduced movement of the effect limb and reduction of the grasp reflex
A constricted left pupil (i.e., miosis) and drooping left eyelid (i.e., ptosis) suggests neonatal Horner syndrome, which can be caused during birth as traction to the brachial plexus damages cervical sympathetic nerve fibers. Klumpke palsy can also result from brachial plexus injury (due to damage of C8 and T1 nerve fibers that supply the ipsilateral intrinsic hand muscles), causing an absent grasp reflex.
trendelenberg sign and what is the cause
what is associated with this in young children
when asked to stand only on one foot, the other sags instead of holding the pelvis linear. this is caused by the active legs superior gluteal nerve damage.
can be caused by misdirected injection of the DTaP vaccine
A retracted opacified tympanic membrane is typically associated with
otitis media with effusion (OME). Although OME is observed frequently in toddlers, it is primarily asymptomatic, since the effusion in the tympanic cavity is characteristically non-inflammatory.
what do you give babies with severe neonatal abstinence syndrome
oral morphine if the presentation is serious.
what do you give babies with mild neonatal abstinence syndrome
supportive care by swaddling, fluids, quiet room
Can babies have neonatal abstinence syndrome from cough syrup
yes
what is a lesser known presentation of hearing impairment
unprovoked episodes of crying and screaming this is usually out of frustration
Do absence seizures have a postictal state
not usually
how long do absence seizures last
usually 5-10 min
what is the characteristic EEG finding for absence seizure
slow spike-wave complexes
A chalazion is a
slowly progressing, firm, rubbery, and nonpurulent nodule on the eyelid. a chalazion is classically a nontender chronic lesion that may present with heaviness of the eyelid.
what is the best way to assess optic glioma
MRI
what is the treatment fort lead exposure
succimer and calcium disodium edetate
what is the presentation of lead toxicity
cognitive delay/decline, microcytic/normochromic anemia, fatigue, constipation, abdominal pain (lead colic), gingival hyperpigmentation (Burton line),
what is the presentation of fanconi anemia
Pancytopenia is also common and presents with mucocutaneous bleeding, recurrent infections, and anemia (normocytic or macrocytic) include short stature, hypopigmentation and hyperpigmentation of the skin, cafe-au-lait spots, microcephaly and developmental delay, ocular abnormalities including esotropia and hypertelorism, and thumb and forearm malformations.
what blood cancers are associated with Fanconi anemia
predisposition for developing acute myeloid leukemia or myelodysplastic syndromes in early adulthood
what stone disorders are common in CF
nephrolithiasis and cholethiasis
what cardiac manifestations can occur in CF
cor pulmonale
what is the presentation of cor pulmonale
right heart failure due to lung disease presents as hepatomegaly, hepatojugular reflux, JVP, pitting edema
what extremity findings are there in CF
clubbing in the fingers, scoliosis and failure to thrive
Long-term intake of certain antiepileptics (e.g., phenytoin) is associated with
a decrease in serum vitamin D levels, hypocalcemia, secondary hyperparathyroidism, and decreased bone density. Rickets is caused due to impaired calcification and mineralization of bones secondary to a disruption in the metabolic pathway of either vitamin D or phosphate.
what is rickets
metabolic abnormality
The initial steps in management of caustic ingestion include
assessment of airway, breathing, and circulation as well as removal of contaminated clothes. Early endoscopy (within 12–24 hours) is important as it allows detailed assessment of the damage to the esophageal lining. The severity of that damage will guide further therapy, such as the need for nasogastric tube placement in case of oropharyngeal injury or, in severe esophageal burns, surgery.
what is the normal physiology of calcium and phosphorus
PTH and vitamin D regulate the uptake of calcium and expulsion of phosphate. if both are elevated simultaneously then there is usually bone breakdown –check alk phos
Oligoarticular juvenile idiopathic arthritis is associated with what ophthalmic disorder
chronic anterior uveitis (often bilateral) in ∼ 25% of cases.
what should juvenile idiopathic arthritis be screened for
anterior uveitis treat asymptomatic anterior uveitis early to help prevent further complications, such as cataracts, iris synechiae, glaucoma, and blindness.
McCune-Albright syndrome presents how
The patient’s triad of polyostotic fibrous dysplasia, café-au-lait spots, and peripheral precocious puberty (due to excess autonomous testosterone production)
An x-ray of her long bones would show the ground-glass appearance of fibrous dysplasia.
Treatment for McCune-Albright syndrome includes
administration of bisphosphonates, surgery of pathological fractures, and administration of antiandrogens and aromatase inhibitors in males.
what happens to the ovaries in turners sydnrome
premature ovarian failure.
what combination of FSH, LH, and estrogen and androgens occurs in Turner syndrome as a result of
The combination of elevated gonadotropin (FSH and LH) levels with decreased levels of estrogen and androgens characterizes hypergonadotropic hypogonadism
what is the treatment for functional constipation
polyethylene glycol for 3-6 days
if persistent try enema
polyethylene can be continued for 2-4 weeks as maintenance therapy
what is the diagnosis for blood-tinged and mucus in the stool without signs of obstruction
usually proctocolitis caused by protein allergy
advise mother to refrain from dairy or soy
what is the treatment for necrotizing enterocolitis s
surgical emergency requiring exploratory laparotomy if there are peritoneal signs
also provide supportive treatment which includes nasogastric decompression
antibiotics broad spec are warranted here
Do we test for rotavirus and does this change management
yes we do and no it doesnt. it is because of the contagiousness that we test. then we can isolate the person
enterobius vermicularis infection presents how and is diagnosed with what test
presents as perianal and perivulvar pruritus with erythema. cellophane tape test to confirm
what is required of post exposure prophylaxis for a child with hepatitis A
nothing. they should be vaccinated
what can prevent pyloric stenosis
breast feeding
what is the most likely underlying cause of intussusception
meckels diverticulum
does a double bubble sign present with epigastric mass
no. double bubble is duodenal atresia and epigastric mass usually pyloric stenosis
Yersinia enterocolitica is primarily acquired through
the consumption of contaminated food, most commonly raw pork. Other documented vehicles of transmission that have been implicated in outbreaks include unpasteurized milk products, unfiltered water, and food contaminated with pet feces. After a typical incubation period of 4–6 days,
Yersiniosis causes
inflammatory diarrhea, nausea, low-grade fever, and, in some cases, RLQ tenderness that may mimic appendicitis (pseudoappendicitis).
Reye syndrome is caused by
hepatic mitochondrial injury that can develop in children 3–5 days after aspirin treatment of a febrile viral illness (most commonly with VZV or influenza B).
Reye syndrome initially presents as
hepatic encephalopathy that manifests with profuse vomiting (due to ↑ ICP) and lethargy, which progresses to delirium, seizures, and coma. Other typical findings include hepatomegaly, elevated AST and ALT (3 times the normal levels), hyperammonemia, hypoglycemia, metabolic acidosis, and a prolonged INR.
what is the most important step in suspected epiglottitis
securing the airway, as this can get worse quickly. diagnostic measures such as x ray are important later on
what is the treatment for mild croup (no intercostal retractions, mild stridor)
cool mist and dexamethasone inhalation
what is the presentation of pertusis
Paroxysmal coughing spells followed by deep, noisy (“whooping”) breaths are very typical of infection with Bordetella pertussis.
treatment of choice for pertussis
what is the post exposure prophylaxis and guidelines
Macrolides (e.g., azithromycin, erythromycin, clarithromycin) They are also given as postexposure prophylaxis to all close contacts of infected individuals, regardless of immunization status.
are macrolide given to babies? what are the risks
when are they given to babies
Macrolides, in general, are not used in infants < 1 month of age because of their adverse gastrointestinal effects; erythromycin, in particular, is associated with an increased risk of infantile hypertrophic pyloric stenosis. However, the potential benefit of avoiding infantile pertussis with azithromycin therapy specifically outweighs the potential harm.
when should adults be vaccinated for pertusis
Regarding vaccination, adults < 65 years of age who have contact with infants should be vaccinated with Tdap to boost potentially waning immunity and prevent potential infection in the infant. Tdap administration is thus important in both the father (unknown immune status) and mother (last booster > 10 years ago).
pertusis infection puts patients at risk of what
due to the coughing and increases in intraabdominal and thoracic pressure can cause epistaxis, abdominal hernias, pheumothorax
also at risk for bilateral conjunctival hemorrhage and petechia i
Croup is considered moderate-to-severe if
the patient has dyspnea and tachycardia at rest, severe stridor with significant retraction, and agitation,
moderate-to-severe croup requires what treatment
nebulizer racemic epinephrine
peritonsillar abscess requires what treatment
I and D and IV antibiotics such as ampicillin and sulbactam
what is the prophylactic treatment for RSV and when is it given and what is its purpose?
palivizumab
given to infants at risk or with brochohlitis and bronchopulmonary dysplasia and premature
this provides passive immunity
what is bronchopulmonary dysplasia
A chronic lung disease primarily found in premature infants exposed to prolonged mechanical ventilation and oxygen therapy (causing barotrauma, oxygen toxicity, and inflammation) for neonatal respiratory distress syndrome.
what is the most common cause of postinfluenza pneumonia
strep pneumonae
this will be a more mild pneumonia than staph and will present without cavitary lung lesions
what is the treatment of post influenza pneumonia
treated empirically with antibiotics (e.g., levofloxacin) until confirmatory data (e.g., cultures) are available.
if someone presents with tonsillitis and the rapid test is negative what is the next step
throat culture
what is the presentation of bronchopulmonary dysplasia
persistent tachypnea, labored breathing (intercostal and subcostal retractions), FiO2 > 30% to maintain peripheral saturation > 90%, and diffuse granular densities with basal atelectasis on x-ray. Later in the disease course, interspersed cystic areas and diffuse hyperinflation of the lung can develop
The first step in management of neonatal respiratory distress syndrome is
nasal CPAP to provide positive end-expiratory pressure. PEEP helps prevent alveolar collapse by providing pressure at the end of expiration when alveoli are most prone to collapse. After initiation of CPAP, an ABG should be obtained to assess the effectiveness of this ventilatory method.
immature neonatal lungs without sufficient surfactant production causes
an increased surface tension in the alveolar sacs that results in alveolar collapse, atelectasis, and ventilation-perfusion mismatch.
If FiO2 > 0.4 or pH < 7.25, after PEEP therapy in neonatal distress syndrome then BLANK is warranted.
intubation and surfactant therapy
why aren’t surfactant therapy and incubation first line therapy for neonatal distress sydnrome
because they have serious complications such as bronchopulomonary dysplasia
what is more specific tuberculin skin test or interferon gamma test
gamma test.
what is the next step in management if a patient has a positive interferon gamma release assay, came from India and has negative chest x ray
isoniazid treatment for 9 months
where is the inflammation characteristic of croup
the subglottal airway
juvenile angiofibroma presents and diagnosed how
as nosebleeds with a pink mass in the nostril. diagnosed with biospy
what is the most common predisposing factor for bacterial sinusitis
viral URI
how does a viral URI predispose to bacterial sinusitis
A viral URTI may cause ostial obstruction and dysfunction of the mucociliary apparatus, leading to the development of sinusitis.
what is the presentation of bacterial sinusitis secondary viral URI
initially clear drainage and mild that prolong and do not improve over the course of 2 weeks, progress to purulent nasal discharge along with facial pain, which indicate secondary bacterial infection.
First-line treatment for uncomplicated acute bacterial rhinosinusitis consists of
amoxicillin-clavulanate.
what is the most common adverse effect of treatment with inhaled corticosteroids.
Oropharyngeal candidiasis (thrush) is the
how to avoid oropharyngeal candidiasis with inhaled glucocorticoid treatment
It can be prevented by gargling with water after use of the inhaler and/or by adding a spacer to metered dose inhalers.
what is the diagnostic test of choice for osteomyelitis and why
bone biopsy. has a high sensitivity and can guide the antibiotic applications
what should precede the first dose of antibiotics for osteomyelitis
bone biopsy. unless acutely ill. broad spec can be given while blood cultures are pending
what is the treatment for a septic joint
prompt synovial drainage via arthrocentesis and antibiotic coverage.
what are the interventions for Legg-calves Perthes disease
mild with no femoral deformity is conservative consisting of limited weight bearing and physical therapy. if there is femoral head deformity then brace and casts.
what is the treatment for slipped cap femoral epiphysis
femoral head pinning
what is the underlying cause of Legg calves perthes
femoral head avascular necrosis
what is the treatment for developmental dysplasia of the hip
harness
what age group is typically affected by Legg-calve-perthes
is most common in children between 4–10 years of age
what age group and body type is typically affected by SCFE
it occurs most often in obese children between 10–16 years of age.
Distal humerus fractures can cause what
Distal humerus fractures, including supracondylar fractures, can lead to arterial compromise either by entrapping or directly injuring the brachial artery.
The genu varum deformity (bow legs) is
normal in children ≤ 2 years and typically corrects as the infant learns to walk and bear more weight on the lower extremities
Seronegative polyarticular JIA is characterized by
the involvement of ≥ 5 joints within 6 months of disease onset. Typical manifestations include symmetrical arthritis of the interphalangeal joints, as well as involvement of the cervical spine and the temporomandibular joint. Chronic anterior uveitis is another common finding.
Systemic juvenile idiopathic arthritis is characterized by
arthritis involving at least 1 joint and the occurrence of systemic symptoms. Joint involvement is most often polyarthritic (may also be oligoarthritic) and commonly affects the knees, ankles, and wrists. Systemic symptoms include intermittent fever and a transient, salmon-pink rash, which often occurs simultaneously to fever spikes. Spleno- or hepatomegaly, generalized lymphadenopathy, and serositis (e.g., pleuritis) can also occur.
Oligoarticular juvenile idiopathic arthritis (JIA) is
the most common type of arthritis seen in children and adolescents. Girls between 2–4 years old are most affected. Oligoarticular JIA presents with asymmetrical arthritis of ≤ 4 large, weight-bearing joints (e.g., knees, ankles) within 6 months of disease onset. The affected joints are often stiff in the morning or after longer periods of inactivity (e.g., sitting), with mobility improving with activity throughout the day. Chronic anterior uveitis is another common symptom and may present with recurring episodes of ocular pain, redness, and photosensitivity. Most patients also have an elevated ESR and increased antinuclear antibody levels but negative rheumatoid factor.
Langerhans cell histiocytosis (LCH) is
a rare disorder that most commonly affects children 5–10 years of age. It presents as single or multiple osteolytic lesions that cause bone pain and swelling. The skull is the most commonly affected bone in children (as in this patient), but LCH can also involve the liver, spleen, and other organ systems.
what is Osgood Schlatter disease, who does it affect and how does it present
Traction apophysitis of the tibial tubercle which typically affects adolescent athletes. Overuse (e.g., sports that involve running and jumping) leads to the patellar tendon exerting excessive strain on the tibial tuberosity, which is not yet fully ossified in adolescents and is thus susceptible to detachment.x-ray shows a separated, sclerotic bone fragment cranial of the tibial tuberosity
first treatment measure in Osgood-Schlatter disease should always be
what are other treatments
a combination of rest, ice, and oral anti-inflammatory medication, like ketorolac. If this combination is not effective, cast immobilization can be attempted. Surgical excision of the fragmented tibial tubercle (and any other ossicles found in the area of the tendon) is reserved for refractory cases.
what is indicated in achondroplasia
neuroimaging to assess the size of the foramen magnum
presentation of achondroplasia
Decreased muscle strength is common in infants with achondroplasia, and in combination with increased deep tendon reflexes, raises suspicion for complications such as brain stem compression and spinal stenosis. Narrowing of the foramen magnum commonly occurs in achondroplasia due to increased growth of the head compared to the torso, which can cause compression of the cervical medulla and lead to subsequent muscular hypotonia/quadriparesis, apnea/hypopnea, and sudden infant death. Neuroimaging is thus indicated at the time of diagnosis to assess the size of the foramen magnum,
Defective type V collagen is the cause of
Ehlers-Danlos syndrome type II (classic EDS),
Ehlers-Danlos syndrome type II (classic EDS), which is characterized by
weakness of connective tissue that predominantly affects the joints and skin. clinical features include joint hypermobility and subluxation (e.g., patella, temporomandibular, shoulder), skin hyperextensibility and fragility, and skeletal abnormalities (e.g., scoliosis). The fragile skin shows a tendency to bruise and break easily, leading to potentially severe lacerations from minor trauma. Cardiovascular features are seen less commonly in classic EDS, but may include heart valve defects and aneurysms/dissections of large arteries.
Defective type III collagen is the cause of
vascular Ehlers-Danlos syndrome
vascular Ehlers-Danlos syndrome, which presents primarily with
cardiovascular manifestations. These include heart valve defects (especially mitral valve prolapse), aneurysms or dissections of large arteries (e.g., aortic, iliac), and berry aneurysms of cerebral arteries. Features of other types of EDS may occur (e.g., skin is fragile, but not extensible), but are rare and are typically much less pronounced. In spite of the fact that cardiovascular symptoms seldom manifest before young adulthood, the severity of this patient’s symptoms cannot be explained by a defect in type III collagen.
spondylolisthesis is
chronic lumbar pain that worsens upon exertion and reclining, a positive step-off sign, a bilaterally positive straight leg raise test, and a waddling gait. Spondylolisthesis is common in all ages. This patient’s history of swimming and weight lifting, both of which involve repetitive hyperextension and rotation of the lumbosacral spine, put him at risk
treatment for spondylolisthesis is
conservative management with physical therapy
is imaging required for the diagnosis of radial head subluxation
no
what is the common presentation of osteogenesis imperfecta
Hearing impairment is a common clinical manifestation of OI, and results from fracture, dislocation, or abnormal formation of the ossicles. also blue sclerae (due to choroidal veins appearing through the overlying thin sclerae), easy bruisability, and brittle, opalescent teeth.
what is recommended for secondary prophylaxis of recurrent acute rheumatic fever in patients with manifestations of carditis but no permanent valvular damage.
Intramuscular benzathine penicillin every 4 weeks for 10 years (or until 21 years of age, whichever is longer)
5 minor criteria of the modified Duke criteria for the diagnosis of infective endocarditis.
These include a predisposing heart condition (ventricular septal defect), fever ≥ 38°C (100.4°F), vascular phenomenon (splinter hemorrhages and Janeway lesions, i.e., purplish lesions on her palms and soles), immunologic phenomenon (glomerulonephritis and Roth spots, i.e, retinal hemorrhages), and a positive blood culture for atypical microorganisms consistent with IE.
what is the best initial test for infective endocarditis
blood cultures
when does rib notching occur in coarctation
not until the age of 5
when does the anterior fontanelle close
around 18 months
when does a ventricular septal defect usually become symptomatic and why
after the first few weeks of life
in utero the pulmonary vascular resistance is high and thus equals the systemic vascular resistance.
epstein anomaly is what
maternal lithium exposure displacement of a tricuspid valve leaflet that results in atrialization of the right ventricle, tricuspid regurgitation, and right atrial enlargementto right atrial enlargement, elongation of the tricuspid valve leaflets, and tricuspid regurgitation,
what are the most common heart defects in downs syndrome
Atrioventricular septal defects (AVSD; also referred to as endocardial cushion defects)
can you treat a VSD with prostaglandin E
no. this worsens the left to right shunt and causes heart failure
what is the treatment for small VSDs
outpatient followup as most of them close spontaneously
what is a common cause of a murmur that goes away when the patient flexes their neck
venous hum. which is caused by turbulence in the internal jugular vein
what is tricuspid valve atresia
this is where is a muscular septum between the right atria and the right ventricle. t
what is the presentation of tricuspid valve atresia
left ventricle hypertrophy, right ventricle hypoplasia, left axis deviation
tetralogy of fallot presents how
most common cyanotic heart defect tet spells of cyanosis. increased cyanosis with valsalva
what are the causes of tetralogy of fallot
usually sporadic, but associated with Downs and DiGeorge
what triggers tet spells and why
crying, feeding, defecation
it activate the the sympathetic nervous system, which triggers spastic contractions of the hypertrophic infundibular musculature (at the transition from the right ventricle to the pulmonary artery).
what do kids with tetralogy usually do
they squat because that increases the vascular resistance and reverses thee shunting
what are VSDs associated with
polycythemia from eisenmengers syndrome (right to left shunting).
Cyanosis shortly after birth that does not improve with supplemental oxygen, and a chest x-ray with the classic egg-on-a-string appearance (i.e., an enlarged heart with narrowed mediastinum due to an overlapping aorta and pulmonary trunk) are suggestive of
a diagnosis of transposition of the great arteries.
what is the cause of a diagnosis of transposition of the great arteries.
maternal diabetes
what is critical for survival in a a diagnosis of transposition of the great arteries.
VSD
what valvular disorders are associated with marfans
aortic regurgitation and mitral valve prolapse
what are the clinical findings for Marfan syndrome heart valve defects
water hammer pulse (aortic regurgitation) click murmur (mitral valve prolapse)
what is the treatment for SVT caused by WPW
procainamide
what medications are contraindicated in WPW (AVRT)
AV nodal blocking agents such as beta blockers, CCB
these will cause the aberrant pathway to become the necessary pathway as there will be less resistance.
what medications are the medications for AVNRT
adenosine, beta blockers, CCB
these will slow the reentry through the circuit. And there is no accessory pathway to worry about.
what is the presentation of hypertrophic cardiomyopathy
systolic ejection murmur caused by the left ventricular outflow track obstruction. rapid squatting softens the murmur while valsalva increases –interventricular petal hypertrophy
what is the cause of death in someone with HCM
arrhythmias
A systolic ejection murmur that increases with valsalva maneuver and standing and decreases with hand grip, squatting, or lying down leads to a diagnosis of what
HCM
what is the treatment of choice for long QT syndrome
beta blockers such as propanolol
what is the treatment of choice for refractory long QT syndrome
implantable defibrillator
what is the presentation of hereditary angioedema
autosomal dominant c1 inihbitor deficiency.
triggers are stress and trauma. results in swelling, erythema without urticaria or pruritus and primarily effects the mucosa of the GI and upper respiratory tract.
episodes are usually self-limiting and resolve within 2–4 days
what is a known risk factor for UTI and why
Constipation is known to be a risk factor for lower UTIs. Prolonged fecal retention leads to bladder dysfunction and urinary stasis, which in turn promotes bacterial overgrowth.
what is the preferred method for getting a urine sample on a child that is not potty trained
transurethral catheterization
why is a renal and bladder ultrasound preformed after a child has a confirmed UTI
to look for anatomical defects and for abscesses
what are the features of posterior urethral valves.
This patient presents with urosepsis, a palpable bladder (midline lower abdominal mass), and a history of oligohydramnios, all of which are features of urinary tract obstruction
Can VUR cause hydronephrosis
yes
what is a complication of vesicoureter reflux
renal scarring
what stone is radioopaque on X ray what shape are the crystals and what test is used to test for it
Hexagon-shaped crystals on urinalysis indicate cystine stones caused by cystinuria. Cystine stones are only poorly radiopaque and often do not appear on x-ray; they are visible on CT, however. Regardless, the positive urine cyanide nitroprusside test confirms the diagnosis.
what are the histological findings for minimal change disease
there is usually nothing which is why it is called minimal change
what is the cause of post strep glomerulonephritis
Poststreptococcal glomerulonephritis is caused by the deposition of immune complexes composed of IgG and C3 complement that contain the streptococcal antigen within the glomerular basement membrane.
what is the course of post strep glomerulonephritis
Recovery of kidney function usually occurs within 6–8 weeks. While 90% of pediatric cases recover completely, approximately 50% of adult patients retain persistently reduced renal function.
recurrent episodes of gross hematuria that is always associated with pharyngitis and with glomerulonephritis glomerular origin of the blood (RBCs with dysmorphic features and RBC casts) suggests what
This combination suggests Berger disease.
what are the features of membraneous neprhopathy
Subepithelial deposits of IgG and complement along the glomerular basement membrane (spike and dome appearance) is the classic finding in membranous nephropathy.
what is a risk factor for membraneous nephropathy
hepatits B infection
Renal papillary necrosis is
what are the common renal findings q
a potentially devastating complication of severe pyelonephritis, which is caused by ascending bacterial infections of the bladder with organisms such as Escherichia coli and other enteric flora. The most common urinalysis findings include hematuria, proteinuria, and pyuria, as seen in this patient.
what is the diagnosis of hypokalemic, hyperchloremic metabolic acidosis with a high urine pH
type 1 renal tubular acidosis decreased activity of the H+/K+ ATPase antiporter on the apical surface of intercalated cell, which reabsorbs K+ and secretes H+ into the lumen of the tubule.
