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most common cause of cerebral palsy?
Is cerebral anoxia
What should you think of in a patient with hypotonia, hyperactive deep tendon reflexes, learning disability with low APGAR scores at birth?
Cererbral palsy most likely 2/2 to cerebral anoxia
what sort of infections do kids with hyper IgM sydnrome get?
recurrent sinopulmonary infections and PCP, opportunistic infections and neutropenia.
characteristics of XLA?
- 1) onset of recurrent bacteria infections in the first 5 days of life
- 2) IgM, G, A are at least 2SD below the normal for age
- 3)poor response to vaccines
- 4)less than 2% CD19+ B cells int the peripheral circulation
what is transient hypogammaglobulinemia of infancy?
extension of hypogammaglobulinemia beyond 6 months of age is termed THI. Usually by 6-11 months of are, dx is based on low level of IgG, normal levels of IgA, and variable levels of IgM.
how to distinguish dx of pyloric stenosis vs GERD?
pyloric stenosis would present earlier in life (3-12 weeks) and it usually leads to projectile vomiting. In GERD frequency of pos-parandial vomiting begins in the first few months of life and peaks around 4months and resolves by about 1 year.
1st line of management is reflux precaution and using thicken formula with rice cereal and upright positioning.
What is Gaucher's disease?
Most common LSD. This is a LSD caused by deficiency of enzyme acid beta-glucosidase. -> anemia, thrombocytopenia, hsm, fatigue and easy bruising, Erlenmeye flask deformity of the distal femur with wrinkled paper appearance of of Gaucher cells in microscopy.
deficiency of sphingomyelinase?
Niemann-pick disease -> FTT, hsm, repidly progressive neurodegenerative usually leading to death by about age 2-3 years.
deficiency of hexosaminidase activity, initially presents as cherry-red spots in the retina and neurologic symptoms in younger age, and later can also lead to hsm.
In case of an epidural hematoma what are some of the criteria that necessitate and emergent carniotomy?
- GCS < 8
- increased ICP
- pupillary abnormalities
- hemiparasis or cerebellar signs
Name some of the findings that you would expect in Kawasaki's disease?
red eyes, high temp, leukocytosis, thrombophilia, erythematous palms with rash that are blanchable, desquamation of the skin is a late finding.
Tx with Aspirin and IVIG
anemia of prematurity is defined by finding the following lab findings:
- 1) peripheral smear shows normocytic, normochromic anemia
- 2) normal total bili levels
- 3) normal WBC and platelets
- 4) low retic count
when do pt with sickle cell get anemic and why not sooner
4-6 months because the HgF protects the cells from sickling
second most common type of muscular dystrophy?
myotonic muscular dystrophy
temporal wasting, inverted V shaped upper lip, all types of muscles are involved (smooth, cardiact, MSK), thenar and hypothenar wasting, cannot relax hand grip.
most common cause of primary amenorrhea?
15 year old comes in with amenorrhea and breast at Tanner stage 2, with decreasesd femoral pulse?
Turner, the decreased emoral pulse is due to coarctation
treatment of choice for impetigo?
usually caused by S. aureus or GAB strep
topical mupirocin or erythromycin PO are drugs of choice
other options include oral dicloxacillin, cephalexin, and ampicillin.
what is the most common type of solid tumors in pediatrics after leukemia?
CNS tumors (2nd most common solid tumors in peds_
about 60% are infratentorial and 25% are supratentorial.
third most common pediatric melignancy?
Neuroblastoma which typically arises in the adrenal glands or sympathetic paraspinal ganglia.
Is Meckel's diverticulum painful or painless?
It results in painless melena in 2-3 year-old-children. Heterotroic gastric tissue may be present in the diverticulum, which is found in the 2-3% of the population, usually about 2 cm long and 2 feet proximal to the ilium.
which one shows up sooner, pyloric stenosis or intestinal atresia?
Intestinal atresia shows up in the first few hours of life whereas pyloric stenosi may present in 2-4 weeks of life.
what will NEC show on radiograph images?
Dilated bowel with pneumotasis intestinalis.
What are some of the complications of acute otitis media?
acute mastoiditis, labyrinthitis, facial paralysis, cholesteatoma, tympanosclerosis, perforation, conductive hearing loss, sensorineural hearing loss.
Treat with a 10 day course of Amoxicillin, alternatives include Amoxicillin-clavulanic acid, cefuroxime, IM ceftriaxone.
Two different types of GBS infection in neonates?
Early-onset: usually presents in the first week of life and typically involves many organs. symptoms include, respiratroy failure, meningitis, DIC, ATN, and peripheral gangrene.
Late-onset: usually presents as more of a focal infection, mostly meningitis (75%), also arthritis, osteomyelitis, cellulitis, UTI.
Pt with recurrent infection (AOM, pneumonia, ... ) and peeling skin on cheecks and feet (eczema), and thrombocytopenia with small platelets?
- WAS (Wiskott-Aldrich Syndrome)
- Which is due to impaired platelet production.
One way to tell the difference between hypothalamic dysfunction leading to percocious puberty and 21-hydroxylase deficiency leading to psudo-puberty
in the HPG there is a less dramatic presentation with sequential development of testicualr enlargement, penis enlargement, pubic hair growth and then growth spurt seen.
Is flexible or rigid bronchoscope for retrieval of foreing body in the lung?
Rigid is for retrieval while flexible is a good diagnostic tool.
nocturnal pain in a child between 2 and 12 that is resolved by the morning and no physical exam finding?
also called congenital hypoplastic anemia in a child with macrocytic anemia, low retic count, and congenital anomalies (cleft lip, webbed neck, shielded chest, triphalangeal thumb).
90% are diagnosed in the first year of life. Usually by 3 months of age.
Treat with corticosteroids, and if too anemic must transfuse.
What are the current guidlines for VZV vaccination post exposure?
Active VZV vaccination for healthy adults and children if 3-5 days post exposure; beyond that it is not that useful.
VZIG is recommended within max 96hrs post eposure for immunocompromised pts
how does the stridor change in laryngomalacia with respect to baby's position?
- It worsens when baby is in supine position. Improved in prone position with neck hyper-extended.
- Also it characteristically worsens with crying.Typically it improves with aging, often subsibes by 18 months of age.
pt between 3-7 yo with acute respiratory distress with stridors, toxic appearing leaning forward, drooling, fever?
- Prepare for intubation and if needed (cannot establish airway) be ready for tracheostomy.
After airway management can start pt on IV Ceftriaxone or Cefotaxime.
What is the treatment for clavicular fracture on a LGA delivery?
Nothing, they heal well on their own.
increase in the levels of HVA and MVA?
Neuroblastoma which arises from neural crest cells. May see calcification of hemorrhage on X-ray and CT
what are some of the findings in acute chest syndrome?
- Fever, chest pain, and infiltrate seen on X-ray.
- This is usually multifactorial is etiology and has been related to pulmonary infarction and infection.
Do you get fever with epiglottitis?
Yes, often pt are are fine before going to bed and wake up wit ha fever, sore throat, dysphagia, muffled voice, drooling and respiratory distress with hyperextension of the neck.
what type of jaundice requires furthre evaluation?
- 1) if it starts in the first 24-36 hrs of life
- 2) if rate of increase is more that 5mg/dL/24hrs
- 3) if total bili is higher than 24 mg/dL in a full term
- 4) if direct bili is greater than 2 mg/dL
Can the painful sicke cell crisis have fever associated with them as well?
Yes they can.
cyanosis in the first few hours of life, no murmur, and a single S2 sound?
Transposition of the great vessels.
Pt with down syndrome and upper motor neuron symptoms should suspect
atlantoaxial instability is seen in 10-15% of these patients
how can you distinguish between autism and asperger?
In asperger kids have normal language development but in other aspect they are similar to autism.
What is Diamond-Blackfen anemia?
This is a pure RBC problem which occurs in the first 3 months of life with normocytic or macrocytic anemia.
how do you diagnose an intusseption?
By barium enema
what is the first line of treatment for strabismus? then what?
First covering the normal eye and forcing the "lazy" eye to correct itself, if this does not work then can do surgical.
what is causative organism for rubella?
- Toga virus
- All females in the childbearing age should get rubella vaccine. However if they are pregnant vaccination should be avoided due to the theoratical risk of infection.
If rubella infx occurs in the first 4 weeks of pregnancy there is a 50% of transmission but in the 3rd trimester there is less than 1% chance of transfer.
what are a few complications of VUR?
60% of kids with VUR also have renal scarring which can lead to HTN (one of the mcc of HTN in kids)
what are some of the findings that you see in Edward's syndrome?
micrognathia, microcephaly, rocker bottom feet, overlapping fingers, absent palmar creases
What is the normal nadir loss in weight?
There about 10% los of weight in the first week or so that should come back after about 10 days.
what is the typicall time frame in which pyloric stenosis can occur?
- 4-6 weeks,
- billious vomiting
can you see osteoprosis with Turner?
Yes, perhaps due to low estrogen there can be osteoprosis .
What are two abd symptoms associated with HSP
abd pain and intussuseption
what do you use in the treatment of tricyclic antidepressant overdose?
sodium barcad is used to correct the acidosis and also helps to narrow the QRS. Can use Benzodiazepine if there is seizure.
What is McCune-Albright syndrome?
This is associated with Cushing's disease but also has Cafe au lait spots, multiple bone defects, precocious puberty.
Marfanoid appearance + thromboembolic events is suggestive of what disease?
Pt with URI treated with ampicillin who develops a polymorphous rash all over the body?
How can you tell the difference between preseptal cellulitis vs orbital cellulitis?
In preseptal there is eyelid discoloration, eyelid swelling, erythema, tenderness however in orbital there is associated visual changes, eye movement problems, proptosis.
How soon after birth is NEC diagnosed and what is seen in physical exam?
- 3-10 DOL
- abdominal distention with gastric residual volume
To prevent pertussis spread is it useful to treat other household members ? with what?
Yes you can treat the household members with Erythromycin.
MCC of nephrotic syndrome in children? and what is it treated with
Minimal change disease, treat with Prednisone for 4-6 weeks. If no response by 8 weeks and hematuria may consider renal biopsy.
How to measure APGAR for a newborn?
Heart, lung, tone, reflex, color
- Heart: >100 is 2 points and <100 is 1 point
- Lung: good effort is 2 point, slow and irregular is 1
- Tone: 2 points for active flextion, 1 for some flextion and 0 for limp
- Color: pink all around 2, body is pink and extremity blue 1, 0 for body and limbs blue
- Reflex: reaction to nasal stimulation; 2 for active cough, 1 for grimance, 0 for no response.
When do you suspect duodenal atresia?
no distension, billious vomiting in the first 24-48 hours, down syndrome, double bubble sign
Why is there jaundice is HUS?
Due to the hemolysis and RBC breakdown
what are 3 ECG findings related to WPW?
- delta wave
- shortened PR interval (since electrical activity travels through the defect quicker and depolarizes the ventricle soon)
- Widened QRS (because of the delta wave)
Some of the associated findings with down syndrome?
- GI: Hirschsprung's disease, pyloric stenosis, esophageal atresia, malrotation of the bowel.
- Heart: Endocardial cushion defect, VSD, PDA
what are two good drugs for treating petit mal seizures?
Ethusuximide and VPA
up to what age is enuresis considered normal? after that age what drug should be used for its treatment
- 4-5 years old
- use DDAVP (drug of choice) or imipramine
What is cyclic vomiting?
- This is intermittant vomiting with no underlying cause that is selflimiting and it is higher in parents that have hx of migrain.
- use of anti-emetic is avices to avoid dehydration
how to distinguish between mongolian spots and bruises?
Mongolian spots are arrested melanocytes so they appear with clear cut edges. They disappear in a few years
Why is PTT high with vit K def related of newborn?
Because vit K acts on II, VII, IX, X, C, S Another name for factor II is prothrombin which is common between both PT and PTT
When do you check for prophobilinogen and aminolevulinic acid?
When suspecting porphorias which would preset with photophobia, neurological symptoms and hyperpigmentation.
Kido with macroglossia, hypoglycemia, hyperinsulinemia, visceromegaly, macrosomia?
A common complication of spondylar fracture in kids?
Brachial artery entrapment. Radial pulse must be checked.
What is a good empiric treatment for pseudomonas infection of say cystic fibrosis?
Double treatment with cephalosporin cefazidime or a ticracillin (pipracillin) + aminoglycoside (gentimicin or amikacin)
in case of forgien body ingestion, do you remove it or not?
If it is beyond the esophagous can consider leaving it in there since about 90% pass uneventfully. However if still in the esophagous can retreive it by endoscopy.
what does a VSD murmur sound like?
pansystolic murumur heard louders at the lower left sternal boarder and can also have diastolic rumbling murumr related to having increased flow through the mitral valve.
Milk protein intolerance?
can present with bloody stool and eosinophils and often a family history of atopy is seen as well.
what type of infection are kids with glactosemia at risk for? and what is the enzyme def. in these kids?
- E. Coli sepsis.
- The enzyme is glactose 1-phosphate uridyl transferase
- They end up presenting with convulsion, hsm, bilateral cateracts, hypoglycemia, mental retardation.
RSV infection in the first two years is a risk factor for developement of .. ..
- it is treated with Ribavirin
Otitis media followed by focal neurological problems with no fever?
Brain abscess should be high on your ddx, sometimes they present with no fever even.
Kopliks spots are pathonomonic for what disease?
Measles caused by Rubeola
HSV-8 causes what dz?
Kaposi Sarcoma seen typically in AIDS
what is the vius that causes Roseola ?
HSV-6 charactirised by 3-4 days of fever and then appearance of a rash that spares the face and once it appears the fever typically ends.
what is the other name for 3-day measles? Does it include the face?
Rubella this rash includes the face
what are the physical manifestations of fetal alcohol syndrome?
epicanthal folds, short palpebral fissure, long philthrum, thin upper lip
How do you treat Lyme disease?
In children less than 9 years of age with Amoxicilling but in >9 yo with Doxycycline. Both for 21 days
most commen manifestation of polycythemia in a newborn?
respiratory distress, poor feedings, and neurological symptoms
How to distinguish betwen Tay Sach's disease and Niemann-Pick's disease?
In Neimann-Pick's disease there is hsm. Tay Sach is due to dif in hexosaminidase while Neimann-Pick is due to dif in sphingomyelinase disease.
What are some of the sx seen in congenital hypothyrodism?
hypotonia, large tounge, weakness, umbilical hernia, sluggish movement, apathy, macrocytic anemia
what is Todd's paralysis?
This is postictal paralysis
What is the most common age range for volvulus?
less than one month
It can be complicated by perforation and peritonitis
Contraindications of breast feeding?
Active TB, HIV, drug abuse