Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Part 2 Paediatrics > Part 2 questions SAQ > Flashcards

Part 2 questions SAQ Flashcards

1
Q

What are the causes of anaemia and jaundice
in neonates

A

first 24 hours, is sepsis until proven otherwise so do full septic screen and start antibiotics (benzlpenicillin and gent)
then in the beginning stages think about haemolysis or sepsis.
Causes of haemolysis: ABO incompatibility, Rhesus incompatibility (decreased incidence due to RoGAM or Rhesus immunoglobulin injection) also think G6DP deficiency
or think about hydrops fetalis.

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

After the first 24 hours Jaundice and anaemia

A

This could be physiological (breastfeeding jaundice/ breast milk jaundice)
hereditary spherocytosis
in certain at risk ethnic groups: sickle cell disease (afro/carribean)
Criggler Najjar
always do conjugated and unconjugated bilirubin high element of conjugated suggest biliary atresia

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

What are the test to look for hereditary spherocytosis

A

FBC looking for anaemia, reticulocyte count can be slightly elevates
peripheral smear microspherocytes, polychromasia
Coombs test: will be negative
osmotic fragility test (but can be falsely normal- if taken during aplastic crisis or recovery from asplastic crisis, or if taken in the neonatal period.
flow cytometry: eosin-5-maleimide binding.

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

What is the treatment for hereditary sphereocytosis?

A

general measures: folic acid supplementation for life
penicillin prophylaxis (if post splenectomy)
vaccination prior to splenectomy (pneumococcal 13 or 25 varient depending on age (less than or greater than 5)
meningococcal vaccination HIB
supportive therapy: Red blood cell transfusions as needed
splenectomy if requiring transfusion or impaired growth
may need cholecystectomy if symptomatic gallbladder disease (due to pigmented stones)

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

Post surgical bleed pethciea and APTT raised factor 8

A

von willebrands disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Von willebrand disease

A

This is an inverted bleeding disorder caused by a deficiency (quantitative or qualitative deficiency ) in von willebrands protein

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

What is von willebrand factor?

A

It is a large multimeric protein that allows platelets to adhere to sites of endothelial injury initiating the primary step in the homeostasis formation of the platelet plug.

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

Which factor is reduced in von willebrands disease

A

it is the VIII (8th) factor because the factor it is a carrier protein.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the different types of von willebrands disease and which are the most common?

A

Type 1: mild to moderate quantitative deficiency (you have working protein but there is just not enough of it) this is the most common. mild bleeding disorder
Type 2: qualitative deficiency, which can be broken down into different subtypes: more severe deficiency
Type 3: near complete quantitative deficiency and this is severe

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

what is the differential diagnosis of von willebrands?

A

primary hemostatic disorders: platelet function abnormalities
congenital thrombocyopenia
mild inherited coagulation factor deficiencies
Hemophillia A
secondary bleeding disorders:
liver disease, uraemia
connective tissue disorders: Ehlers danlos and scurvy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What other disorders can prolong the APTT but not cause bleeding

A

factor XII deficiency

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

what is the treatment of von willebrands disease

A

desomopressin it stimulates the release of von willebrand factor from the endothelial cells. However it’s use is limited to type 1 as it only helps in a quantitative reduction in von willebrand and some of the type 2 (contraindicated in type B)
factor concentrates humate P.
recombinant von willebrand factor (only in severe disease)
transexamic acid

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

what are three non infectious causes of petechiae

A

acute lymphoblastic leukaemia
idiopathic thrombocytopenia purpura
henoch schonlein purpura

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

what is Diabetic ketoacidosis

A

An emergency presentation commonly in type 1 diabetics. Defined as:
1. Acidemia PH less than 7.3
2. Hyperglycemia: greater than 11, glycosuria
3. ketonuria/ ketosis (greater than 3)
4. bicarbonate less than 18

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

What are the calculations for a child with fluids in a child with DKA

A

The following of the fluid calculations are important as they can lead to cerebral oedema if not managed correctly

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

what are the goals of DKA management

A

correct and reverse the ketosis
correct dehydration
restore glucose to normla
monitor for complications of DKA and it treatment
identify and create any precipitating event

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

What are the steps in fluid resuscitation

A

if the child is volume deplete but not shocked give one fluid bolus (10ml/kg) with NACL 0.9%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

How do you calculate the percentage dehydration?

A

mild: just clinically detectable
moderate: 5% dry mucus membranes and reduced skin turgor
severe: (10%) sunken eyes and poor cap refill
shock: tachycardia

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

What are the criteria for ICU admission in DKA

A

PH less than 7.1
hyperventilation, shock, low GCS, persistent vomiting, inability to sustain own airway, age less than 2

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

how do you calculate the maintenance

A

requirements= maintenance plus deficit
deficit is calculated by multiplying (wt in Kg) by (% either 0.03,.05,.08) the MAX you can multiply is 0.08! even if you calculate the child at 10% deficit
there is a chart to calculate ml/kg/24 hour depending on the weight
so first you need to calculate the maintenance which is weight multiplied by the (chart number)
Then you want to multiply that by 2 to get what you need over the next 2 days
then you want to multiply the weight but percent deficit as a decimal multiply by 1000.
then add those two number and subtract the fluid bolus given (10 times weight)
then put that total over 48. This will get your rate for the next two days.

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

What type of fluids would you use in DKA

A

first normal saline
then once the patient is passing urine or the gas shows not hyperkalemia and ECG t waves normal add 20mmol KCL into the bag. (you are monitoring this every 2 hours)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

When do you need to start adding glucose to the bag

A

you are going to check the the glucose every hour, you want a decrease of 4-5 mol per hour
if it is more than this rate, add dextrose 5%
if the glucose is between 14-17

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

How to calculate insulin infusion rate for children over 5 kg

A

0.1 mL/kg/hr
continue until the PH is greater than 7.3
discontinue 30 minutes after the first subcutaneous injection is given.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

what kind of monitoring does a child in DKA need

A

continuous ECG monitoring
half hourly: neuro obs
hourly: vital signs and blood glucose and ketones measurements
2 hourly: electrolytes and calculate the corrected NA/ VBG and lab glucose until academia is reduced.
review the fluid composition
twice daily weights

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

Should you immediately start insulin therapy?

A

no you must wait 1 hour after the fluid bolus is given! Not waiting has been shown to increase the risk of cerebral oedema.

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

what is a neuroblastoma

A

tumour arising from neural crest cells of the sympathetic nervous system.
normally presents in children less than 5 years old
midline hard tumour arising from the midline.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what are the investigations for a neuroblastoma

A

urine catecholamine metabolics (raised homovanillic acid and vanillylmandelic acid)
CT/MRI
tissue biopsy to confirm disgnosis, prognosis and therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

how is a neuroblastoma determined to be benign or malignant (prognosis)

A

pathological or molecular features (histological characteristics, tumour priody, MYCN amplification, 1p, and 11q23 status

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

what is the treatment for a neuroblastoma

A

low risk: surgery plus or minus chemotherapy
high risk: multiple cycles of chemotherapy and autologous bone marrow transplant, cisretinoic acid, immunotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What are the red flags for sitting independently

A

9 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

what are the red flags for not walking by

A

18 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

when should you definitely have 2 word sentences

A

2 year s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

when should you have toilet training by

A

5 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What is congenital adrenal hyperplasia

A

it is a series of autosomal recessive disorders that are classified by having a common deficiency in the biosynthesis of cortisol.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What is the common cause of congenital adrenal hyperplasia

A

21Ohase deficiency

36
Q

what is non classic congenital adrenal hypertrophy

A

it is when there is a late onset presentation of virilasation

37
Q

what is the most common presentation of congenital adrenal hyperplasia

A

most likely to be a salt wasting crisis

38
Q

how does classic CAH present in females

A

androgen excess during the early prenatal life resulting in ambiguous genitalia
present with adrenal crisis

39
Q

how does classic CAH present in males

A

progressive varilization and salt wasting crisis

40
Q

what is strabismus

A

any form of ocular misalinement

41
Q

amblyopia

A

is a decrease in best corrected visual acuity in an otherwise anatomically normal eye

42
Q

refractive error

A

improper focusing of light by the eye which generally requires correction by eyeglasses or contact lenses

43
Q

myopia

A

neaersightness

44
Q

hyperopia

A

farsightedness

45
Q

astigmatism

A

unequal curvature of the cornea causing the cornea to be more curved in one direction than the other.

46
Q

What is Eisenmenger syndrome

A

If a large VSD is left untreated pulmonary vascular disease with eventually develop leading to reversal of the hunt, cyanosis and RV failure.

47
Q

how do you calculate the anion gap

A

Anion gap= NA + K - CL -HCO3

48
Q

Maternal Hep B

A

There are three core antigens
and also IgG and IgM (M for occurred in last few months).. G as in long gone (more than 6 months ago). HBsAG is highly contagious also will measure there viral load DNA as well)

49
Q

what do you treat babies with exposure to Hep B mums

A

IVIG and Hep B vaccination

50
Q

For babies with hep B exposure

A

they are followed in the in Rainbow clinic (infectious disease clinic) in 6 months
to asses whether or not immune they look at the surface antigen negativity and anti HBS positivity

51
Q

How do you diagnose spinal muscular atrophy

A

This is progressive disorder of the motor neurones in the spinal cord and brain stem.

52
Q

what is a differential diagnosis for SMA

A

prader willi syndrome, congenital myasthenia gravis

53
Q

one of the treatments for SMA

A

nusinersen, PT and OT, spinal fusion surgery,

54
Q

complications of SMA

A

respiratory failure,
scoliosis
limitations in mobility due to muscle wasting.

55
Q

Which genetic syndrome is associated with brachycephaly

A

Apert syndrome

56
Q

subacute combined degeneration of the cord definition

A

this is characterised by degeneration of the dorsal columns and the lateral columns of the spinal cord due to demyelination

57
Q

What does subacute combined demyelination of the cord present with

A

sensory deficients, parasethesia, weakness, ataxia, and gait disturbance.

58
Q

What are the causes of subacute combined demyelination of the cord

A

B12 deficiency (in children who are vegan or picky eaters)
malabsorption (Chron’s disease)
use of proton pump inhibitors

59
Q

what is used for the newborn hearing screen

A

automated otoacoustic emissions test

60
Q

What if the baby fails the first hearing test

A

the next test will either be a second automated otoacoustic emissions test or they will use a automated auditory brainstem response test

61
Q

what is the treatment for sensory neural hearing loss

A

cochlear implant

62
Q

what is the congenital infection associated with sensory neural hearing loss

A

cytomegalovirus

63
Q

What if a child presents with sensory neural hearing loss and a goitre

A

this is a sign of pondered syndrome. This is the most common syndromal form of deafness. It is associated with developmental abrnomalities of the cochlea, sensorineural hearing loss and diffuse thyroid enlargement.

64
Q

child presenting with fever weight loss and pleural effusion differentials

A

lymphoma, and parapneumonic effusion

65
Q

Whats is viral myositis

A

can happen in the context of a viral infection like influenza, also check the urine if positive for myoglobinuria consider rhabomyolyis which requires hospitalisation for IV hydration therapy.

66
Q

A 12 year old male presents with raised MBI new onset hip pain and shortened and external rotated femur

A

this fits with slipped upper femoral epiphysis
the confirmatory test is to do a plain film of the hips in AP and frog leg lateral view

67
Q

What are the X-ray findings in slipped upper femoral epiphysis

A

Klein’s line (evaluation of asymmetry)
the S sign
blurring of the proximal femoral metaphysics (blanch sign of steel)

68
Q

What are three predisposing factors of slipped upper femoral epiphysis

A

obesity, history of radiation to the femoral head region, and elevated leptin levels

69
Q

What is the management of slipped upper femoral epiphysis

A

surgical correction ie: percutaneous in situ fixation

70
Q

what are you thinking of an neonate with a pleural effusion on TPN

A

Traumatic chylothorax: injury to the thoracic duct (post cardiac surgery)
catheter related aetiologies: extravasation of the IV fluids into the thoracic cavity
venous hypertension from intravascular blood clots resulting in pleural effusions
hemothorax: hemorrhagic disease of the newborn, DIC, vascular malformations
sepsis

71
Q

a neonate has elevated triglycerides in the analysis of his pleural effusion following his diagnosis of a pleural effusion what is the cause?

A

this is consistent with a chylothorax, this type of effusion is a exudative effusion.

72
Q

What are the two types of effusions

A

transudative and exudative

73
Q

What is the management of a chronic pleural effusion?

A

chest drain for slow continuous drainage of fluid using an underwater seal system
replacement of losses (with enteral and TPN)
possibility of octreotide (after careful discussion with parents about risks and benefits- specialist consultant input)

74
Q

What are the X-ray findings in rickets

A

fraying, splaying and cupping of the metaphyseal ends

75
Q

what are the causes of rickets

A

dietary: protracted exclusive breastfeeding without supplementation
Lack of sun exposure
prematurity

76
Q

What are the two subcategories of rickets

A

calcipenic rickets and phosphopenic rickets

77
Q

What are the lab findings of calcipenic rickets

A

PTH: markedly elevated
calcium low or normal
phosphate: low or normal

78
Q

what are the lab findings in phosphopenic rickets

A

PTH: normal or modestly elevated
calcium: normal
phosphate: low

79
Q

what are the causes of hypophospatemic rickets

A

hereditary hypophosphataemic rickets (vitamin D resistant rickets)
falconi syndrome

80
Q

What are the goals of treatment in metabolic conditions

A

to suppress toxic metabolites
correct hypoglycaemia
correct the acidosis (bicarbonate administration)
correct the hyperammonia (suppress protein metabolism)

81
Q

What are the labs going to look like in a urea cycle defect

A

PH is going to be alkaline
the anion gap, ketones, glucose, and lactate are going to be normal. The ammonia with be greatly elevated

82
Q

What are the labs going to look like in adrenal insufficiency

A

electrolytes: hyponatriema and hyperkalemia and acidosis
hypercalcemia
hypoglycaemia

83
Q

What are the criteria for neurofibromatosis

A

six or more cafe au last spots measuring at least 5mm in size before puberty and 15 mm in size after puberty
freckles in the axilla or groin
two or more neurofibromas or more plexiform neurofibroma
lisch nodules
optic glioma
tibial dysplasia or abnormality of the orbit
first degree relative with the same diagnosis

84
Q

how many of the criteria need to be fulfilled to diagnose NF1

A

at least two features

85
Q

what is the HEADSS assessment

A

Home
Education
Activites and Alcohol
Diet and Drugs

Self-esteem and sex
safety and suicide

86
Q

intussusception definition

A

invagination or telescoping of a proximal portion of the bowel into the distal segment of the bowel, which can result in schema or obstruction

87
Q

What is your first line treament when you suspect intussusception

A

keep nil by mouth, start Iv fluids and get an urgent surgical consult
if heamodynmically unstable then they are not for reduction by enema

Part 2 Paediatrics (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions