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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Normal genu varum Cxs?

A

Symmetric
Normal stature
No leg discripancy
No lateral thrust while walking

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Rett syndrome pathophysiology?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

additional finding?

A
Seizure
Respiratory difficulty
Head growth restriction
Sleeping problem
Autistic behavior
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

CXS?

A

Hypogonadotropic hypogonadism
Delayed puberty
Anosmia/Hyposmia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

delay puberty in females?

A

> = 12 year

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

osteoid osteoma pathogenesis?

A

Benign bone-forming tumor

MC in adolescents boys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

X-ray?

A

Small round lucency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Managment?

A

NSAID
Serial x-ray
Surgery for refractory case

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

neuro complication of meningitis?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

risk factor?

A

young age
high bacterial load
prolonged seizure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what to do?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What px neded in patient with SCD?

A

Twise dailey prohlactic penicilin untill age 5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Deficiet in social comunication and intraction Cxs?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Restricted,repititive pattern of behaviour?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Managment?

A

Compresive(Sp,Beh,edu service)

Pharmacotherapy for concomitant psychiatric illness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Transient protinuria?

A
Fever
Stress
Exersise
Seizure
Volume deplesion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

orthostatic protinria?

A

adolecent boys

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Constitusional delay of growth and puberty?

A
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

How to d/t with familial short stature?

A

Short family
Not reach adult hight
but may have normal velocity

27
Q

In GH deficiency?

A

short stature
Delay in growth velocity
delay in bone age

28
Q

In gonadal?

A

Normal bone age

29
Q

Nutritional?

A

wight also affected

30
Q

Managment of CDGP?

A

Reassurance

+/- Hormonal tx

31
Q

febrile seizure diagnostic criterion?

A

Age 6m-5year
No px febrile seizure
No metabolic derangement
No sign of CNS inf

32
Q

managment?

A

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
Q

Abnormal sexual behavior in children?

A

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
Q

sign of sexual abuse?

A

Anogenital trauma
Coercive sex act
Repeated UTI

35
Q

empiric managment of epiglottitis?

A

Ceftriaxon(HIB,NTHI,SPy,Spnu )

Vancomycine(MRSA)

36
Q

slipped capital femoris CM?

A

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
Q

Parader will syndrome genetics?

A

maternal Uniparental disomy Cr 15

Due to deletion/mutation in paternal cr.a5

38
Q

Parader will syndrome CM?

A
hypotonia
weak sucking
excessive feeding
obesity
short stature
hypogonadism
Intellectual disability
facial dysmorphism
39
Q

facial dysmorphism?

A

Narrow forehead
Almond-shaped eye
Downturned mouth

40
Q

Complication?

A

Sleep apnea
T2DM
Gastric distension/rapture
Death by choking

41
Q

Diamond black fan anemia?

A
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
Q

Managment?

A

Corticosteroid

Transfusion

43
Q

food-induced allergic proctocolitis pathogenesis?

A

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
Q

CM?

A

Painless bloody stool

+/- spit-up

45
Q

managment?

A
Dietary modification(infant and mother)
Resolve by itself after 1 year
Use hydrolyzed(metabolized protein)formula
46
Q

backwith-widman syndrome pathogenesis?

A

deregulation and impaired gene expression in 11p15

gene encodes ILGF2

47
Q

CM?

A

Macrosomia and fast growth until late childhood
macroglossia
Midline defect(Umbilical hernia and omphalocele
Himihyperplasia

48
Q

complication?

A

Wilms tumor
Hepatoblastoma
Neonatal hypoglycemia(hyperinsulinism)

49
Q

Surveillance?

A

Serum alpha protein
renal U/S
RBS

50
Q

pathogenesis of phenylketonuria?

A

Phenyl hydroxylase deficiency
Autosomal recessive
Failure to convert phenylalanine to tyrosine

51
Q

CM?

A

Musty body odor
Seizures
Severe intellectual disability
Hypopigmentation involving skin,hair,eyes, and brain Nuclei

52
Q

Diagnosis and managment?

A
Newborn screening(tandem mass screening)
quantitative amino acid analysis(phenylalanine)
Dietary restriction of phenylalanine
53
Q

IDA and PLT count?

A

IDA cause reactive thrombocytosis

54
Q

What to do if parents refuse to vaccinate their children whether due to personal or religious beliefs?

A

Respect their decision
Explain on benefits of vaccination
A document that you explained

55
Q

Acute rhinosinusitis suspect bacterial?

A

Fever > 3 day
New/Recurrent fever after initial improvement
Persistent system >= 10 day

56
Q

Viral RhinS?

A

No/early fever resolution
Mild facial pain/tenderness
Improvement & resolution by day 5-10

57
Q

General symptoms?

A

Nasal congestion &/or purulent discharge
Facial pressure/pain
Cough, headache,loss of smell and ear pain

58
Q

Managment?

A

Intranasal saline/irrigasion by saline
NSAID
Antibiotic if bacterial

59
Q

What to do in patients with pyloric stenosis?

A

First, correct DHN and electrolyte loss before surgery(Reduce post-operative apnea risk and overall outcome)

60
Q

what to suspect if the patient develops ecchymosis and haemarthrosis with minimal trauma?

A

Suspect coagulation disorder

61
Q

Managment of SCA maintenance?

A

Vaccination
Penicillin until age 5
Folic acid suplementation
Hydroxyurea if have an indication

62
Q

What are the indications for hydroxyurea?

A

Recurrent pain crisis
History of acute chest syndrome
Sever symptomatic anemia

63
Q

Mechanism?

A

Reduce SCHb percent and increase FHb —Reduce pain crisis by decreasing vascular obstruction.

64
Q

Managment of acute pain crisis?

A

Hydration
Analgesia
+/ transfusion