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Pead 7 Flashcards

1
Q

Normal leg alignment in devt?

A

6 mon–Genu varum
2 year–Straight leg
4 year—Gnu Valgum
> 7 years —Strait’s leg

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2
Q

Normal genu varum Cxs?

A

Symmetric
Normal stature
No leg discripancy
No lateral thrust while walking

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3
Q

Rett syndrome pathophysiology?

A

Loss of neurodevelopment due to dendrite formation in cortex defect and neuromodulation
X-linked
MECP2 gene defect

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4
Q

Key feature?

A 
Present 6-18 month
Usually females
Normal development then regression
Loss of speech
Gait abnormality
Loss of purposeful hand movement
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5
Q

additional finding?

A 
Seizure
Respiratory difficulty
Head growth restriction
Sleeping problem
Autistic behavior
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6
Q

Kallman syndrome genetics and pathogenesis?

A

X-lR/AD or AR in wommen
46 XY/XX chromosome
Failure of the olfactory and GNRH producing neuron migration—prosencephalon hypoplasia and Central hypogonadism

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7
Q

CXS?

A

Hypogonadotropic hypogonadism
Delayed puberty
Anosmia/Hyposmia

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8
Q

delay puberty in females?

A

> = 12 year

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9
Q

sign of puberty?

A 
Growth of pubic hair, other body hair, and facial hair.
Enlargement of testicles and penis.
Muscle growth.
Growth spurt.
Acne.
Deepening of the voice.
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10
Q

osteoid osteoma pathogenesis?

A

Benign bone-forming tumor

MC in adolescents boys

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11
Q

CM?

A

The proximal femur is MC site
Leg pain(worse at night, Relived by NSAID and not related to activity)
No systemic symptoms
Tenderness, Swelling, and deformity

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12
Q

X-ray?

A

Small round lucency

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13
Q

Managment?

A

NSAID
Serial x-ray
Surgery for refractory case

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14
Q

neuro complication of meningitis?

A

intellectual and behavioral disability
hearing loss( chochlial infn–fibrosis and calcification)
seizure
cerebral palsy

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15
Q

risk factor?

A

young age
high bacterial load
prolonged seizure

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16
Q

what to do?

A

audiometry screening(asymptomatic early and difficult for tx after ossification)

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17
Q

What px neded in patient with SCD?

A

Twise dailey prohlactic penicilin untill age 5

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18
Q

CM of autism spectrum disorders?

A

Deficiet in social comunication and intraction
Restricted,repititive pattern of behaviour
-/+language and intelectual impairment
Changing to new things induce anxiety

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19
Q

Deficiet in social comunication and intraction Cxs?

A

Sharing of emotional interest
Difficulty in verbal communication
Develop and understand relation

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20
Q

Restricted,repititive pattern of behaviour?

A

Repititive movt/speche
Insistanse on samness/routiness
Intense fixated interest
Adverse responce to sensory imput

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21
Q

Managment?

A

Compresive(Sp,Beh,edu service)

Pharmacotherapy for concomitant psychiatric illness

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22
Q

asymptomatic isolated protinuria aroch?

A

Do first morning urine Pr/Cr ration
If normal:transient/orthostatic protinuria
If high evaluate for glomerular/parenchymal illnes

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23
Q

Transient protinuria?

A 
Fever
Stress
Exersise
Seizure
Volume deplesion
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24
Q

orthostatic protinria?

A

adolecent boys

standing(at end of day + ve and -ve at morning)

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25
Constitusional delay of growth and puberty?
``` family Hx Delay growth b/n 6m-3Y Short stature(<2SD) Normal growth velocity >= 3 year Delay in bone age(Cxs) Delay in puberty Reach normal adult hight lately ```
26
How to d/t with familial short stature?
Short family Not reach adult hight but may have normal velocity
27
In GH deficiency?
short stature Delay in growth velocity delay in bone age
28
In gonadal?
Normal bone age
29
Nutritional?
wight also affected
30
Managment of CDGP?
Reassurance | +/- Hormonal tx
31
febrile seizure diagnostic criterion?
Age 6m-5year No px febrile seizure No metabolic derangement No sign of CNS inf
32
managment?
abortive(>5min) fever tx reassure and discharge(risk of rec in first 24 hr is low) educate a family(High risk of feature FS and 15 epilepsy)
33
Abnormal sexual behavior in children?
precocious sexual knowledge object insertion to the anal, vagina, or oral area using force, bribe, and aggression in a sexual act preoccupied with masturbation
34
sign of sexual abuse?
Anogenital trauma Coercive sex act Repeated UTI
35
empiric managment of epiglottitis?
Ceftriaxon(HIB,NTHI,SPy,Spnu ) | Vancomycine(MRSA)
36
slipped capital femoris CM?
MC in adolescents Unable to weight-bearing and climbing Unable IR, Abd, and Flexion of hip Pos and inferior displaced femoral head and sup, anteriorly displaced femur
37
Parader will syndrome genetics?
maternal Uniparental disomy Cr 15 | Due to deletion/mutation in paternal cr.a5
38
Parader will syndrome CM?
``` hypotonia weak sucking excessive feeding obesity short stature hypogonadism Intellectual disability facial dysmorphism ```
39
facial dysmorphism?
Narrow forehead Almond-shaped eye Downturned mouth
40
Complication?
Sleep apnea T2DM Gastric distension/rapture Death by choking
41
Diamond black fan anemia?
``` pure red cell progenitor defect low RBC half-life Low HCT, Reticulocyte Macrocytic anemia Normal platelet and WBC Craniofacial anomaly Triphalengial thumb High risk of malignancy ```
42
Managment?
Corticosteroid | Transfusion
43
food-induced allergic proctocolitis pathogenesis?
family/personal hx of allergy, asthma, and eczema Non-IgE mediated allergically proctocolitis Occur in infants feed milk protein or in breastfeed mother usage of milk and soy proteins
44
CM?
Painless bloody stool | +/- spit-up
45
managment?
``` Dietary modification(infant and mother) Resolve by itself after 1 year Use hydrolyzed(metabolized protein)formula ```
46
backwith-widman syndrome pathogenesis?
deregulation and impaired gene expression in 11p15 | gene encodes ILGF2
47
CM?
Macrosomia and fast growth until late childhood macroglossia Midline defect(Umbilical hernia and omphalocele Himihyperplasia
48
complication?
Wilms tumor Hepatoblastoma Neonatal hypoglycemia(hyperinsulinism)
49
Surveillance?
Serum alpha protein renal U/S RBS
50
pathogenesis of phenylketonuria?
Phenyl hydroxylase deficiency Autosomal recessive Failure to convert phenylalanine to tyrosine
51
CM?
Musty body odor Seizures Severe intellectual disability Hypopigmentation involving skin,hair,eyes, and brain Nuclei
52
Diagnosis and managment?
``` Newborn screening(tandem mass screening) quantitative amino acid analysis(phenylalanine) Dietary restriction of phenylalanine ```
53
IDA and PLT count?
IDA cause reactive thrombocytosis
54
What to do if parents refuse to vaccinate their children whether due to personal or religious beliefs?
Respect their decision Explain on benefits of vaccination A document that you explained
55
Acute rhinosinusitis suspect bacterial?
Fever > 3 day New/Recurrent fever after initial improvement Persistent system >= 10 day
56
Viral RhinS?
No/early fever resolution Mild facial pain/tenderness Improvement & resolution by day 5-10
57
General symptoms?
Nasal congestion &/or purulent discharge Facial pressure/pain Cough, headache,loss of smell and ear pain
58
Managment?
Intranasal saline/irrigasion by saline NSAID Antibiotic if bacterial
59
What to do in patients with pyloric stenosis?
First, correct DHN and electrolyte loss before surgery(Reduce post-operative apnea risk and overall outcome)
60
what to suspect if the patient develops ecchymosis and haemarthrosis with minimal trauma?
Suspect coagulation disorder
61
Managment of SCA maintenance?
Vaccination Penicillin until age 5 Folic acid suplementation Hydroxyurea if have an indication
62
What are the indications for hydroxyurea?
Recurrent pain crisis History of acute chest syndrome Sever symptomatic anemia
63
Mechanism?
Reduce SCHb percent and increase FHb ---Reduce pain crisis by decreasing vascular obstruction.
64
Managment of acute pain crisis?
Hydration Analgesia +/ transfusion

Pediatrics (15 decks)

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