Paeds additional Flashcards

1
Q

what is Hirschsprung’s disease

A

aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses.

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2
Q

condition associated with hirschsprung’s disease

A
  • downs syndrome (MC)
  • neurofibromatosis
  • Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)
  • Multiple endocrine neoplasia type II
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3
Q

presentation of hirschsprung’s disease

A
  • Delay in passing meconium (more than 24 hours)
  • Chronic constipation since birth
  • Abdominal pain and distention
  • Vomiting
  • Poor weight gain + failure to thrive
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4
Q

Mx of Hirschsprung’s disease

A
  • Ix= AXR and rectal biopsy
  • initially: rectal washouts/bowel irrigation, fluid resus, IV abx
  • definitive management: surgical removal of affected segment of the colon
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5
Q

Features of innocent murmurs

A

ALL S
- Soft
- Short
- Systolic
- Symptomless
- Situation dependent (the murmur gets quieter with standing, or only appears when the child is unwell or feverish)

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6
Q

pansystolic murmurs

A
  • Mitral regurgitation, heard loudest in the mitral area
  • Tricuspid regurgitation, heard loudest in the tricuspid area
  • Ventricular septal defect, heard loudest at the left lower sternal border
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7
Q

causes of ejection systolic murmurs

A
  • Aortic stenosis
  • Pulmonary stenosis
  • Hypertrophic obstructive cardiomyopathy
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8
Q

Heart defects that can cause a right-to-left shunt, and therefore cyanotic heart disease, are:

A
  • ASD
  • VSD
  • PDA
  • Transposition of the great arteries
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9
Q

Causes of anaemia in infancy

A

Physiologic anaemia = MC

The other causes of anaemia in infants are:
- Anaemia of prematurity
- Blood loss
- Haemolysis
- Twin-twin transfusion

Haemolysis is a common cause of anaemia in infancy. There are a number of causes of haemolysis in a neonate:
- Haemolytic disease of the newborn (ABO or rhesus incompatibility)
- Hereditary spherocytosis
- G6PD deficiency

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10
Q

what is physiological anaemia of infancy

A

normal dip in haemoglobin around 6-9 weeks of age in healthy term babies

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11
Q

causes of anaemia of prematurity

A
  • Less time in utero receiving iron from the mother
  • RBC creation cannot keep up with rapid growth in the first few weeks
  • Reduced erythropoietin levels
  • Blood tests remove a significant portion of their circulating volume
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12
Q

test to check for immune haemolytic anaemia

A

direct Coombs test

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13
Q

causes of anaemia in older children

A
  • iron deficiency
  • blood loss

Rarer causes
- sickle cell
- Thalassaemia
- Leukaemia
- Hereditary spherocytosis
- Hereditary eliptocytosis
- Sideroblastic anaemia

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14
Q

Symptoms specific to iron deficiency anaemia:

A
  • Pica = dietary cravings for abnormal things such as dirt
  • Hair loss
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15
Q

Sx of anaphylaxis

A
  • Urticaria, itching, Angio-oedema, abdo pain, swelling around lips and eyes
  • SOB
  • Wheeze
  • Stridor
  • Tachycardia
  • Lightheadedness
  • Collapse
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16
Q

how to confirm anaphylaxis

A

mast cell tryptase within 6hrs

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17
Q

signs and sx of appendicitis

A
  • centro abdo pain then RIF pain
  • McBurney’s point (1/3 ASIS to umbilicus)
  • N&V
  • fever
  • guarding and rebound tenderness
  • Rovsing’s sign
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18
Q

mx of acute asthma

A
  1. oxygen <94%
  2. SABA (nebulised mod-severe, spacer mild-mod)
  3. nebulised ipratropium bromide
  4. oral prednisolone, IV if unable to swallow
  5. oral LTRA
  6. nebulised magnesium sulphate for severe asthma

Can consider
7.IV salbutamol
8. IV aminophylline
9. IV magnesIum sulphate
CALL SENIOR

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19
Q

How to diagnose asthma

A

> 5 y/o spirometry
FeNO
Peak Flow
Direct bronchial challenge test

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20
Q

steroids for eczema (mild, moderate, potent)

A
  • Mild: Hydrocortisone 0.5%, 1% and 2.5%
  • Moderate: Eumovate (clobetasone butyrate 0.05%)
  • Potent: Betnovate (betamethasone 0.1%)
  • Very potent: Dermovate (clobetasol propionate 0.05%)
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21
Q

most common organism in bacterail infection of eczema

A

staph aureus

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22
Q

what is eczema herpeticum caused by

A

herpes simplex virus (HSV) or varicella zoster virus (VZV)

HSV1 most common

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23
Q

presentation of eczema herpeticum

A

widespread, painful, vesicular rash
+ systemic symptoms such as fever, lethargy, irritability and reduced oral intake.
There will usually be lymphadenopathy

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24
Q

what is biliary atresia

A

section of the bile duct is either narrowed or absent –> cholestasis –> accumulation of conjugated bilirubin

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25
Q

presentation of biliary atresia

A
  • jaundice due to high conjugated bilirubin levels shortly after birth lasting >14 days post birth
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26
Q

Ix for biliary atresia

A

conjugated and unconjugated bilirubin (can conj but just can’t excrete it)

27
Q

what is bronchiolitis caused by

A

Respiratory syncytial virus (RSV)

28
Q

presentation of bronchiolitis

A
  • <1 yr, usually under 6 months
  • particularly in ex-premature babies with chronic lung disease.
  • Coryzal symptoms: VRUTI
  • Signs of respiratory distress
  • Dyspnoea
  • Tachypnoea
  • Poor feeding
  • Mild fever (under 39ºC)
  • Apnoeas
  • Wheeze and crackles
29
Q

signs of respiratory distress

A
  • tachypnoea
  • head bobbing
  • nasal flaring
  • use of accessory muscles
  • intercostal and subcostal recessions
  • tracheal tugging
  • cyanosis
  • abnormal airway noises
30
Q

difference between wheeze, grunting and stridor

A
  • Wheezing= whistling sound caused by narrowed airways, during expiration
  • Grunting= exhaling with the glottis partially closed to increase PEEP
  • Stridor= high pitched inspiratory noise caused by obstruction of the upper airway e.g. croup
31
Q

progression of bronchiolitis

A

coryzal sx day 1-2
worst sx day 3-4
then improve, lasts 7-10 days

32
Q

when to admit with bronchiolitis

A
  • < 3months
  • 50 – 75% reduced feed
  • Clinical dehydration
  • RR > 70
  • O2 < 92%
  • Moderate to severe respiratory distress
  • Apnoeas
  • Parents not confident in their ability to manage at home
33
Q

prevention of bronchiolitis

A

Pavilizumab monthly injection

34
Q

Causes of cerebral palsy

A

Antenatal:
- Maternal infections
- Trauma during pregnancy

Perinatal:
- Birth asphyxia
- Pre-term birth

Postnatal:
- Meningitis
- Severe neonatal jaundice
- Head injury

35
Q

which parts of the brain are affected by different types of Cerebral palsy

A

Spastic- UMN
Dyskinetic- Basal Ganglia
Ataxis- Cerebellum

36
Q

signs and sx of cerebral palsy

A
  • Failure to meet milestones
  • Increased or decreased tone, generally or in specific limbs
  • Hand preference < 18 months = KEY
  • Problems with coordination, speech or walking
  • Feeding or swallowing problems
  • Learning difficulties
37
Q

complications of cerebral palsy

A
  • Learning disability
  • Epilepsy
  • Kyphoscoliosis
  • Muscle contractures
  • Hearing and visual impairment
  • Gastro-oesophageal reflux
38
Q

Causes of constipation

A
  • Functional
  • Cystic fibrosis
  • Hirschsprung’s disease
  • Hypothyroidism
  • Spinal cord lesions
  • Sexual abuse
  • Intestinal obstruction
  • Anal stenosis
  • Cows milk intolerance
39
Q

age group for croup

A

6months- 2 years

40
Q

cause of croup

A

parainfluenza virus

41
Q

sx of CF

A
  • meconium ileus
  • failure to thrive
  • chronic cough
  • thick sputum
  • recurrent resp infections
  • steatorrhoea
  • child salty when parent kisses
  • abdo pain and bloating
  • finger clubbing
  • nasal polyps
42
Q

how to diagnose CF

A
  • Newborn blood spot testing
  • The sweat test is the gold standard. Chloride conc >60mmol/L
  • Genetic testing for CFTR gene
43
Q

key colonisers in CF

A

Staphylococcus aureus- MC
Haemophilus influenza
Klebsiella pneumoniae
Escherichia coli
Burkhodheria cepacia
Pseudomonas aeruginosa- important

44
Q

colonisers that exempt pt with CF from lung transplant

A
  • burkholderia cepacia
  • ?mycobacteria abscessus
45
Q

conditions screened for in CF

A
  • diabetes
  • osteoporosis
  • vitamin D deficiency
  • liver failure
46
Q

Mx of CF

A
  • CFTR modulators (Kaftrio)
  • chest physio
  • vaccines (pneumo, influ, varicella)
  • Nebulised DNase
  • Creon
  • high calorie diet
  • prophylactic fluclox
  • lung transplant
47
Q

complications of Down syndrome

A
  • Learning disability
  • Recurrent otitis media
  • Deafness. Eustachian tube abnormality–> glue ear and conductive hearing loss.
  • Visual problems such myopia, strabismus and cataracts
  • Hypothyroidism
  • Cardiac defects
  • Atlantoaxial instability
  • Leukaemia
  • Dementia: Alzheimer’s
48
Q

tests for Downs syndrome

A
  • combined test @ 11-14 weeks (high NT, high b-HCG, low PAPPA
  • triple test @ 14-20 weeks (high b-HCG, low AFP, low serum oestriol)
  • quadruple test @ 14-20 weeks (+ high inhibin
49
Q

tests if high risk of Downs syndrome

A
  • CVS
  • amniocentesis
  • NIPT
50
Q

cause of epiglottitis

A

haemophilus influenza type B

51
Q

presentation of epiglottitis

A
  • Sore throat and stridor
    -cDrooling
  • Tripod position
  • High fever
  • Difficulty or painful swallowing
  • Muffled voice
  • Scared and quiet child
  • Septic and unwell appearance
52
Q

Mx of epiglottitis

A
  • secure the airway
  • leave child alone
  • IV abx (e.g. ceftriaxone)
  • dexamethasone
53
Q

what is Henoch Schonlein Purpura

A
  • IgA vasculitis
  • purple rash on bottom and legs
  • IgA deposits in the blood vessels
  • affects skin and GI tract
54
Q

features of Henoch Schonlein Purpura

A
  • Purpura (100%),
  • Joint pain (75%),
  • Abdominal pain (50%)
  • Renal involvement (50%)
55
Q

causes of hyposplenism

A
  • splenectomy
  • sickle-cell
  • coeliac disease, dermatitis herpetiformis
  • Graves’ disease
  • systemic lupus erythematosus
  • amyloid
  • splenic sequestration crisis can lead to splenic infarction, leading to hyposplenism
56
Q

features of hyposplenism

A
  • Howell-Jolly bodies
  • siderocytes
57
Q

what is juvenile idiopathic arthritis (JIA)

A

arthritis without any other cause, lasting more than 6 weeks in a patient under the age of 16

58
Q

subtypes of JIA

A
  1. Systemic JIA
  2. Polyarticular JIA
  3. Oligoarticular JIA
  4. Enthesitis related arthritis
  5. Juvenile psoriatic arthritis
59
Q

systemic JIA

A
  • Still’s disease
  • Subtle salmon-pink rash
  • High swinging fevers, weight loss
  • Lymphadenopathy, splenomegaly
  • Joint inflammation and pain
60
Q

complication of systemic JIA

A

macrophage activation syndrome (MAS) =
- severe activation of the immune system + massive inflammatory response.
- acutely unwell child + DIC, anaemia, thrombocytopenia, bleeding and a non-blanching rash

61
Q

polyarticular JIA

A
  • 5 joints or more
  • symmetrical
  • mild fever, anaemia and reduced growth
  • equivalent to RA in adults
62
Q

oligoarticular JIA

A
  • 4 joints or less
  • affects larger joints, usually monoarthritis
  • <6 y/o girls
  • anterior uveitis
  • ANA positive
63
Q
A