Pediatrics 7,9,10 Flashcards
What should be suspected in a baby with DiGeroge syndrome
- low serum calcium levels
- get echocardiography
CATCH for DiGeorge
- Conotruncal cardiac defects
- Abnormal facies
- Thymic aplasia
- Celft palate
- Hypocalcemia
In patients with hypertrophic cardiomyopathy, maneuvers that increase preload or afterload
- squatting
- leg raise
- hand grip
Maneuvers that decrease LV cavity by decreasing preload
- Valsalva
- abrupt standing
- amyl nitrite administration
hypertophic cardiomyopathy is common in who and how is it inherited
African American
Autosomal Dominant
Appears in age less than 1 with respiratory ( stridor, wheezing, coughing) and/or esophageal (dysphagia, vomiting, difficulty feeding) symptoms. Stridor is biphasic. Improves with neck extension
vascular ring
Laryngomalacia
collapse of the supraglottic structures during inspiration and presents with chronic inspiration stridor that improves with prone positioning
Pansystolic murmur that is loudest at the left lower sternal border and a diastolic rumble at the apex due to increased flow across the mitral valve
large ventricular septal defect
a 2 hour child with single loud second heart sound has what wrong with them
- single loud second heart sound
most common congenital cyanotic heart disease in the neonatal period
D-transposition of the great vessels
x-ray finding for D-transposition of the great vessles
“egg on a string” ( narrow mediastinum)
Beck’s triad
- distant heart sounds
- distended jugular veins
- hypotension
Trisomy 18 baby with murmur at left lower sternal bordner
VSD
Most common heart defect in down syndrome
complete atrioventricular septal defect
what is an innocent cardiac murmur
- intesnity is grade I or II
- decreases with standing
what maneuvers reduce the intensity of innocent murmurs
decrease venous blood return ( standing, Valsalva)
patients with prolonged QT intervals are at risk for
- syncope
- ventricular arrhythmias
- sudden cardiac death
Those with congenital QT prolongation should avoid
- electrolyte derangements
- medications that block potassium channels
how do you prevent cardiac arrest in prolonged QT syndrome
beta blockers with pacemaker
greater than 5 day fever, bilateral nonexudate conjunctivitis, mucositis, cervical lymphadenopathy, rash and extremity changes
kawasaki
treatment for kawasaki
aspirin and intravenous immunoglobulin
Most serious potential sequelae of Kawasaki
coronary artery aneurysms
hear a harsh III/VI holosystolic murmur, next step
- echocardiography, not reassurance because it is harsh III/VI
can viral myocarditis cause liver problems
yes, hepatomegaly
Difficulty swallowing, feeding refusal and vomiting should raise concern for
foreign body ingestion
what ethnicity has decreased UGT activity
asians
child with impaired adaption to darkness, photophobia, dry scaly skin, xerosis conjunctive, sources cornea keratomalacia, bitot spots and folllicular hyperkeratosis of shoulders, buttocks, and extensor surfaces
vitamin A deficiency
white baby with growth failure and recurrent sinopulmonary infections typically raise concern for
cystic fibrosis
pancreatic insufficiency in baby can lead to
- steatorrheahea
- failure to thrive
- vitamin deficiencies
Common associations with celiac disease
- type 1 diabetes
- dermatitis herpetrifromis
how can celiac disease present
- weight loss
- iron deficiency anemia
the only absolute infant contraindication to breastfeeding is
galactosemia
Classic signs of older children with biliary cyst
abdominal pain, jaundice and may have pancreatitis
what should be suspected in all children with recurrent intussusception
Meckel’s diverticulum
Triple bubble sign on x-ray and gasless colon
Jejunal atresia
risk factors for jejunal atresia
- exposure to cocaine and other vasoconstrictive drugs
what is the workup if a baby has bilious emesis
- abdominal x-ray then contrast enema
electrolyte values after prolonged vomiting
- hypochloremic
- hypokalemic
- metabolic alkalosis
next step of management if something is in esophagus
removed immediately
what should be suspected in a newborn with feeding intolerance, abdominal dissension and bloody stools
necrotizing enterocolitis
risk factors for necrotizing enterocolitis
- prematurity
- hypotension
- congenital heart disease
hallmark x-ray finding for necrotizing enterocolitis
pneumatosis intestinalis
in child, recurrent self-limiting episodes of vomiting and nausea without an apparent cause suggets
cyclical vomiting
someone with cyclical vomiting my have a family history of what
migraine
what should be suspected when a well-appearing neonate has painless bloody stools
milk or say-protein proctocolitis
newborns with conjugated hyperbilirubinemia and hepatomegaly
biliary atresia
painless hematochezia in a young toddler is most likely due to
Mecke’s diverticulum
how is Meckel’s diverticulum diagnosed
technetium-99th pertechnetate scanning
what is gold standard for diagnosing malrotation
upper gastrointestinal contrast
what syndrome has an increased risk of Alzheimer and hypothyroidism
Down syndrome
Choanal atresia can be part of what sydnrome
CHARGE
what does CHARGE stand fro
- Coloboma
- Heart Defects
- Atresia
- retardation of growth/development
- Genito-urinary anomalies
- ear
Moderate to severe dehydration in children should be treated with an
intravenous bolus of isotonic fluid
what is major protein in human milk
whey: more easily digested than casein and whey helps to improve gastric emptying
what is usually elevated in during pregnancy for gastroschisis
increase AFP
infant with macrosomia, macroglossia, umbilical hernia/omphalocele, hemihyperplasia, and hypoglycemia
Beckwith-Wiedemann syndrome
Patients with Beckwith-Wiedmann syndrome are at significantly increased risk for what
- Wilms tumor
- hepatoblastoma
how do you screen for Beckwith-Wiedmann syndrome
- abdominal ultrasound and alpha-fetoprotein levels
what is first-line modality in diagnosing intussusception
ultrasound
how do you reduce obstruction of intusseception
air enema
Increased gastric residual volume, vomiting, and abdominal distension in a preterm neonate are highly suspicious of
necrotizing enterocolitis
X-ray finding for necrotizing enterocolitis
Pneumatosis intestinalis and portal venous air
Niacin ( vitamin B3) deficiency causes
- pellagra: diarrhea, dermatitis, dementia, and possibly death
Angular cheilitis, stomatitis, glossitis, normocytic-normochromic anemia- and seborrheic dermatitis
riboflavin B2 deficiency
angular cheilitis
fissures at corners of lips
excess pyridoxine can cause
peripheral neuropathy
Trichotillomanis has high comorbidity with
OCD
what group of kids (2) are at increased risk of precocious sexual development
- obese children
2. black or hispanic ethnicities
Isolated premature-adrenarche is a significant risk factor for
PCOS
type 2 diabetes
metabolic syndorme
When should vaccines be given to preterm infants
- chronologic age rather than gestational age
what is the qualifting factor for first Hepatitis B vaccine to be given
weight should be greater than 2 Kg ( 4lb 6oz)
Metatarsus adductus? txt?
congenital foot deformity in which the forefoot turns inward
- resolves spontaneously
hydroxyurea benefits patients with sickle cell by
increasing fetal hemoglobin
a girl with foul-smelling vaingal discharge and vaginal spotting or bleeding? txt?
vaginal foreign bodies
- calcium alginate swab or irrigation with warmed fluid
sickle cell patient with sudden drop in hemoglobin with a very low reticulocyte count and lack of hepatosplenomaegaly?
aplastic crises
New-onset hearing loss or chronic ear drainage despite antibiotic therapy are typical presenting symptoms of ?
cholesteatomas
symptoms of iron poisoning ? X-ray findings? txt?
- abdominal pain
- hematemesis
- metabolic acidosis
- radiopaque on x-ray
- Deferoxamine
vaso-occulsion of the bone in sickle cell can cause
osteonecrosis
mutation in type 1 colllagen
osteogenesis imperfecta
define polycythemia in term newborn
hematocrit greater than 65 percent
risk factors for polycythemia
- cord clamping
- maternal hypertension
- maternal diabetes mellitus
symptoms for polycythemia in term newbron
- respiratory distress
- hypoglycemia
- neurologic
most common cause of urinary tract obstruction in newborn boys
posterior urethral valves
Can an 18th month old run or walk independently
yes
gold standard for confirmation of muscular dystrophy is
genetic testing
Most common cause of breast mass in adolescent
fibroadenoma
adolescent patient with suspected fibroadenoma should be examined when
after menstrual period for decrease in mass size or tenderness
how should you work up NK1
MRI
how is myotonic muscular dystrophy inherited
autosomal dominant
grip myotonia, facial weakness, foot drop, dysphagia, and cardiac conduction anomalies. Cataracts, testicular atrophy/infertility and balness
myotonic muscular dystrophy
treatment for central precocious pubety
- always get CT or MRI
- GnRH analog therapy
uncomplicated acute bacterial rhinosinusitis should be treated with
oral amoxicillin-clavulanic acid
differential diagnosis of a solitary, painful, lytic long bone lesion with overlying swelling and hypercalcemia in child
Langerhans cell histiocytosis
increasing head circumference and signs of increased intracranial pressure in children should be evaluated how?
CT scan of brain
Most common type of brain tumor in children
astrocytoma
contraindications for DTP vaccine
- immediate anaphylaxis
- unstable neurologic disorders
- encephaolopathy within a week
first line treatment for seborrheic dermatits
emollients and nonmedicated shampoos
