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Pediatrics > Pead3 > Flashcards

Pead3 Flashcards

1
Q

Glucose-6-Phosphatase deficiency?

A 
Glycogene -1-storage(Von Gierke disease)
Typically present 3-4 month age
Due to the absence of enzymes in the Liver, Kidney, and intestine
Hypoglycemia
Hepatomegaly
Lactic acidosis
Hyperuricemia
Hyperlipidemia
Doll like face
Thin
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2
Q

Cause of asplenia?

A

Anatomic(Surgery, absence)

Functional(autoasplenia due to fibrosis(SCD),Infilitrative disease(sarcoidosis),Chongestion(PHTN)

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3
Q

Complication?

A

High risk of encapsulated organism asplenia

Holy jolly bodies(precipitated nuclei remaining)

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4
Q

What about Heinz’s body?

A

Seen in G6PDH deficiency
Is precipitated Hb
Seen by Special stain(Crystal Violate), unlike HJB which can be seen by wright stain.

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5
Q

What about basophilic striping?

A

Multiple, blue-green pigment on pheriphery

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6
Q

Sign of hypotonia in Down Syndrome?

A

protruding tongue
flaccid abdomen
Loss of extremity flexion(slip from examiner’s hand)

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7
Q

Visual acuity testing In children?

A

All children age 3-4
Snell chart
HOTV or LEA(Pic) in not lerned child

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8
Q

All other tests should be done routinely?

A

Red reflex testing(cataract & retinoblastoma)
Corneal reflex(alignment)
Cover test for old children to assess strabismus

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9
Q

Indication for the referral?

A 
Pupil asymmetry >1 mm
<20/40 at age 4 and <30/40 in 5 and above
Nystagmus
Ptosis
other conditions obstruct vision
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10
Q

Autism screening?

A

at 18 month/2 year

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11
Q

Depression?

A

at age of 12

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12
Q

Laryngomalacia?

A

A common cause of stridor in infants.

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13
Q

CM?

A

Inspiratory stridor
Increase inspiration(feeding,crying) exacerbate stridor
GERD feature
Omega shaped epiglottis

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14
Q

management?

A

mainly resolve by 18 month

Surgery indicated in case of RD, Cyanosis, and failure to thrive

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15
Q

Stridor types and cause?

A

supraglottic lesion–Inspiratory S
Subglottic lesion–Biphasic
Tracheal(Intratoracic airway)–Expiratory

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16
Q

Henoch-Schonelin purpura pathogenesis?

A

IgA mediated leukocytoclastic vasculitis

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17
Q

CM?

A 
Palpable purpura
Arthritis/Arthralgia
Abdominal Pain
Intussusception
IgA nephropathy similar renal feature
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18
Q

Lab?

A 
Normal platelet and coagulation
Normal/Raised Cr.
haematuria
\+/- proteinuria/RBC cast
Nephritic/nephrotic syndrome in adult
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19
Q

Managment?

A

Suportive(fluid and NSAID)

admission and systemic CS in sever case

20
Q

Kawasaki disease complication?

A

Aneurysm–thrombosis–MI

21
Q

Chronic granulomatous disease feature?

A

X-linked recessive
Recurrent pul. and skin infection
Catalase producing bacteria(S,A ,burkloderia,seratia and aspargilus)

22
Q

Diagnosis?

A

DHR test

NT test

23
Q

How about Hyper IgE(Job syndrome)?

A

may have rec skin abscess and pul infc. but the patient will have facial deformity(broad nose) and bone abnormality(scoliosis) and strong family Hx(AD)

24
Q

TODD paralysis?

A 
Occur in post ectal phase
Flaccide weakness/paralysis 
Involve one/both extremities in one side
due to nuronal exusion
CT recommended to r/o other serious cause
The seizure may not be witnessed
25
Q

Heamoglobine concetration in normal individual?

A

HgA–95-98
HgA2-2.5%
FH–<1 %
SSH–Absent

26
Q

HgA1?

A

Thalassemia Mi/SST–Low

Thalassemia Mj/SSD–Absent

27
Q

HgA2?

A

Hihg in Thalassemia mi/Mj

Normal in SSD/SST

28
Q

HgF?

A

Near normal in Thalasemia Min/SST

High in Thalasemia Maj/SSD

29
Q

SShb?

A

high in SSD/SST but absent in thalassemia

30
Q

Thalassemia major cxs and Tx?

A

hemolytic Anemia
Splenomegaly
treat by frequent transfusion and chelation for iron overload

31
Q

Cause of pneumothorax in neonates?

A

HMD
MAS
Due to rapture

32
Q

Managment?

A

If life-threatening emergency needle thoracotomy
Continue If pat on CPAP
Reduce PEEP if on MV

33
Q

What to do inpatient with hypospadias?

A

leave circumcision
karyotype-Asses mosaicism
renal U/S(asses internal genitalia)

34
Q

what is associated with it?

A

Undescended tests
Cordea
Conjecital hernia

35
Q

effect of maternal estrogen on neonates?

A

Breast enlargement
Luckorachia
minimal Vx bleeding(withdrawal)
Labia enlargement

36
Q

Managment?

A

Transient

Not need workup

37
Q

spontanious Pnumomediatrnum CM?

A 
Acute chest pain
SOB
Cough
Subcutaneous emphysema
Crunching sound over the heart
38
Q

diagnosis?

A

C-X-ray

39
Q

managment?

A

rest
analgesics
avoid valsava

40
Q

Risk factor?

A

RTI
asthma exacerbation
Tall, thin adolescent

41
Q

mickle diverticulum symptoms?

A

asymptomatic
lower GI bleeding
Anemia

42
Q

complication?

A

Volvulus
Intussusception
Obstruction

43
Q

Diagnosis and managment?

A

TEC scan

Surgery if symptomatic

44
Q

Etiology of galactosemia?

A

AR

Due to GALT deficency

45
Q

CM?

A 
Cataract
Hepatomegaly
Jaundice
Vomiting and failure to thrive
Hypoglycemia
High risk of E.Coli sepsis
46
Q

Lab?

A

Bilirubin elev.
Metabolic acidosis
Low glucose
+ urine reducing substance

Pediatrics (15 decks)

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