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Paediatrics > FOP > Flashcards

FOP Flashcards

1
Q

What feature is mostly suggestive of “Orbial Cellulits”

A

Restricted Eye Movements:
suggests involvement of the orbital septum indicating deeper involvement.

Tx : IV-antibiotics
Imaging: CT / MRI Head

Refer:
-> Ophthalmology
-> ENT

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2
Q

S/E: Medications in Breast Milk:

1) Tetracycline
2) Isoniazid
3) Chloramphenicol

A

1) Yellow Teeth, avoid in Breastfeeding, or Age <12

2) Increased Seizure/Convulsion Risk
Peripheral Neuropathies in Newborn

3) Bone Marrow Toxicity in Infants
“Grey Baby Syndrome”
- abdominal distension
- cyanosis
- circulatory collapse (rare)

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3
Q

TRUE OR FALSE:

Examination of the Throat is essential in Children that present with Stridor ?

A

FALSE:

Throat examination should be avoided in children presenting with Stridor, as this can provoke or worsen upper airway obstruction.

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4
Q

Signs of Foreign-body inhalation ?

a) Clinical
b) CXR

A

ASYMMETRY

a) Asymmetrical Chest Expansion or Wheeze

b) Asymmetrical Hyper-expansion of Lung Fields

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5
Q

What is Kartagener Syndrome ?

A

Subset:
Primary Ciliary Dyskinesia

Characteristic Triad:
1) Bronchiectasis
2) Chronic Sinusitis
3) Situs Inversus

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6
Q

What are the Stages of Clubbing ?

A

1) Fluctuant Nail-bed

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7
Q

SIDS Advice:

A

1) Temperature 16-20 oC
2) Supine on their Back
3) Same room (reduces SIDS 50%)
4) Avoid Co-sleeping: Bed / Armchair
5) Cot or Moses basket free from Toys
6) Smoke-FREE

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8
Q

Cholera Vaccination Regime [NICE] ?

A

2-6yrs: 3 doses [1-6 wks apart]

6-17yrs : 2 doses [1-6 wks apart]

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9
Q

What type of Vaccine is the Cholera Vaccine?

A

Dukoral - inactivated oral vaccine

(rCTB) - recombinant cholera toxin B
4 types of Killed V.Cholerae

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10
Q

1st Line Invx for Hypertension in Kids?

A

Ambulatory BP-monitoring (ABPM)

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11
Q

What are the characteristic features of Noonan’s Syndrome:

A

“male-TURNERS / psuedo Female-TURNERS)

Dystrophic Features:
- Hypertelorism
- Downslanting palpebral fissures
- Webbed Neck
- Short Stature

Cardiac Abnormalities (90%)
- Pulmonary Stenosis
- Hypertrophic Cardiomyopathy [HCM]

Skeletal defects

Crytorchidism

Bleeding diathesis (tendencies)

Learning Difficulties

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12
Q

Inheritance of Noonan’s syndrome?

A

Autosomal-DOMINANT

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13
Q

How does Cystinosis present?

A

Cystinosis: Autosomal Recessive [AR]
Lysosomal recessive disease

  1. Nephropathy
  2. Poor Growth
  3. Hypophosphataemic Rickets
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14
Q

What is the characteristic triad for hypospadius?

A

1) Foreshortened Ventral opening
2) Chordee [Ventral Tilt]
3) Hooded foreskin

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15
Q

Neonate with Double Bubble Sign on AXR:

A

Duodenal Atresia:
- More prevalent in T21 [Down’s Syndrome]
- Bilious Vomiting from 1st feed (< 24hrs)

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16
Q

How does SMA - Type 1 present at brith?

A

“Werdnig-Hoffman disease” - Most Severe Spinal Muscular Atrophy

  • Hypotonia
  • Respiratory insufficiency
  • Feeding difficulties
  • Alert expressionless face,
  • Weakness (Proximal > Distal)
  • Deep tendon reflexes absent or depressed
  • Tongue fasciculations
  • Limb deformities caused by intrauterine hypotonia
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17
Q

Day 5 Heel Prick:

A

1) Glutaric acid Uris type I
2) Isovaleric acidaemia
3) MCADD
4) Phenylketonuria
5) Cystic Fibrosis
6) Congenital Hypothyroidism

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18
Q

What is VACTERAL?

A

Vertebral Anomalies
Anorectal malformation
Cardiac defects
Tracheo-oesophageal fistulae
Renal anomalies
Limb deformities

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19
Q

Anorectal - Urethral Fistualae with Anorectal malformation

A

1) De-functoning colostomy
2) Micturating cyst-urogram
3) Primary anoplasty

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20
Q

Grades of IVH:

A

Grade - 1 : Subependymal IVH (germinal matrix) - good prognosis + long term outcomes requires monitoring
Grade - 2: IVH -/- ventricular dilation
Grade - 3: IVH + Ventricular dilation -> Hydrocephalus + Contralateral impairment
Grade - 4: Parenchymal -> Hydrocephalus [requiring VP-shunting]

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21
Q

SMA-Type 1:

A

Autosomal Recessive [AR]
Deletion of SMN-gene Ch 5q
Severe form presents in the neonatal period with:
- Severe Hypotonia
- Feeding difficulties
- Respiratory distress

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22
Q

Ladds Procedure:

A

Correction of Malrotation - widening of the mesenteric base

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23
Q

What is Choanal Atresia?

A

Congenital blockage of the Nasal passages, which can occur unilaterally or bi-laterally
- Unilateral: Recurrent Nasal Drainage,
Recurrent Sinus infections
- Bilateral: Resp.Distress + Cyanosis

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24
Q

Congenital Myotonic Dystrophy [DM1]

A

Genetics: CTG - Trinucleotide repeat in non-coding region DMPK

Antenatal Presentation: Polyhydramnios, reduced Fetal movements -> Failure to Progress

Neonatal Presentation:
Respiratory distress
Profound hypotonia
Facial Diplegia
Minimal Suckle
Athrogyroposis (Talipes)

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25
Q

Haematology Bloods in DIC:

A

Depletion of Plts + VWF + Clotting Factors:

Affects both Primary + Secondary Haemostasis

(H) Bleeding Time [Thrombin Time] - common pathway
(H) PT [Prothrombin Time] - extrinsic pathway
(H) aPTT [activated partial thrombin time] - intrinsic pathway

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26
Q

Haematology bloods in Haemolytic Newborn Disease [HND]:

A

Due to Vitamin K deficiency / depletion: - affects VIT.K dependent clotting factors in common + extrinsic + intrinsic pathway: [II, VII, IX + X]

(N) Thrombin Time + Fibrinogen levels
(H) PT - [VII]
(H) aPTT - [IX]

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27
Q

Haematology bloods in Haemophilias ?

A

Intrinsic pathways are affected -> reduced 2o Haemostasis
1) A - VIII (8)
2) B - IX (9)
3) C - XIII (13)

(N) - Bleeding time
(N) - PT
(H) - aPTT

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28
Q

At what gestation does Surfactant get produced ?

A

24 - 28 wks

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29
Q

In what conditions can Exopthalmos Present ? [4]

A

BWS - Beckwith-Wiedemann Syndrome

T21

T18

Pentalogy of Cantrell

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30
Q

Clinical Features: [5]
Beckwith-Wiedemann Syndrome [BWS]

A

Rare - genetic condition

1) Exomphalos
2) Macroglosia
3) Visceromegaly
4) Pancreatic hyperplasia - > (H) Insulin production ->
recurrent hypoglycaemia
5) Genitourinary abnormalities

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31
Q

What disease is the biggest risk factor for fungal pneumonia?

A

Chronic granulomatous disease;

X-linked recessive (CYBB gene)

Inability of phagocytes to kill and engulf organisms due to defective enzymes.

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32
Q

What is the most likely Dx?

Teenage boy presents with a Hx of ‘blocked nose, pink/blue mass, unilateral nostril.

Hx of Trauma —> profuse blood loss

A

Juvenile Angiofibroma

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33
Q

What are the characteristics features of
Tuberous Sclerosis Complex?

A

[AD] multisystem disease - Hamartomas

  • Brain —> Seizures [80%]
    Infantile Spasms [50%]
  • Heart
  • Lungs
  • Skin
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34
Q

What is Precocious Puberty?

A

Early pubertal development:

Girls < 8yrs (commonly idiopathic)

Boys < 9yrs (commonly pathological)

35
Q

What is the average age of Breast development “Thelarche” in girls?

A

11 years

(Absence by 13yrs is considered delayed)

36
Q

What are the 2 main causes of Acquired Hypothyroidism?

A
  1. Autoimmune (Hashimoto’s) Thyroiditis
  2. Post-Total-Body-Irradiation (TBI)
    - e.g. post bone-marrow biopsy
37
Q

Klinefelter Syndrome, disease associations?

A

Pulmonary Disease
Varicose Veins
Breast Cancer
Leukaemias
Mediastinal germ-cell tumours

38
Q

What is the Prevalence of Delayed Puberty ?

A

More common in boys, usually idiopathic

39
Q

What are the affected organs in multiple endocrine neoplasia [MEN1]

A

Pancreatic Insulinoma

Pituitary Tumour

Parathyroid Hyperplasia —> Parathyroidism
- (H) Ca2+ increased bone mobilisation
- (L) PO4- increased renal excretion

FHx: Maternal Prolactinoma

40
Q

What structure is most likely to be affected by suprasellar extension of a Pituitary Adenoma?

A

Optic Nerve (Optic Chiasm) —> Bitemporal Hemianopia

41
Q

What is McCune-Albright Syndrome?

A

Mutations in GNAS gene Ch20 13.3

Fibrous Dysplasia
Cafe Au Lait macules
Autonomic endocrine hyper-function:
- Gonadotropin-independent precocious
puberty
- Hyperthyroidism
- Hypercorticolism
- Acromegaly

42
Q

What is the most common cause of Cardiac defects in Children?

A

VSD

  • Alchohol increases risk of septal defects *
43
Q

CXR sign “egg on string”, with Cyanosis with reduced pre+post ductal sats?

A

TGA

44
Q

Able to roll, babbling, reaching for objects, sit with support?

A

6 months

45
Q

Able to roll, not reach for objects

A

4 months

46
Q

Sit-unsupported

A

9 months

47
Q

2 word sentences
Use a fork
Stack x6 blocks

A

2 yrs-old

48
Q

3 word sentences
Know own name
‘Tri’-cycle

A

3 yrs

49
Q

Balance on 1 foot
Brush his teeth independently
Write his name
No nappies (dry day & night)

A

5 yrs-old

50
Q

Use scissors & buttons
Not dry @ night
Not be able to spell name

A

4 years

51
Q

Lyme disease treatment

A

Amoxicillin

52
Q

< 3months old Bacterial meningitis management?

A

IV-Cefotaxime

(Ceftriaxone can exacerbate hypebilirubinaemia in <3 months —> Kernicterus)

53
Q

Sepsis management?

A

Cefotaxime/Ceftriaxone(hyperbilirubinaemia risk)

Amoxicillin / Ampicillin < 28 days

Gentamicin [Severe]

± Aciclovir

54
Q

What are the features of congenital Parvovirus (B19) ?

A

Non-immune Hydrops Fetalis

Miscarriage (9-20wks)

Severe Anaemia

Cardiac Failure (Viral Myocarditis)

Non-blanching rash

Extramedullary Haematopoesis

55
Q

Signs of Congenital CMV? [5]

A

IUGR

Microcephaly

Petichial/purpuric rash

Hepatosplenomegaly

Peri-ventricular calcification

Cranial - ventriculomegaly

Chorioretinitis / Cataracts

Sensorineural Deafness

56
Q

Signs of Congenital HSV [3]?

A

Microcephaly

Cutaneous Scars

Vesicles

57
Q

Signs of Congenital Rubella? [6]

A

Cataracts

Cardiac Defects:
- PDA
- Pulmonary Artery Stenosis

B/L SNHL

Hepatosplenomegaly

Microcephaly

Petechiae

58
Q

Signs of Congenital Syphilis? (8)

A

Coryzal

Hepatosplenomegaly

Maculopapular rash - palms + soles

Hydrops

Vesicles

Condylomata Lata

Bone Abnormalities

Pseudoparalysis

59
Q

Signs of Congenital Toxoplasmosis? [4]

A

IUGR

Hydrocephalus

Intercerebral calcification

Chorioretinitis

60
Q

Signs of Congenital Zika Virus? [5]

A
  1. Microcephaly
  2. Intracranial calcification
  3. Ocular abnormalities
  4. Hypertonia
  5. Joint Contractures [Arthrogyposis]
61
Q

Sign’s Cow’s milk protein allergy ?

A

Non-IgE mediated: Symptoms can occur 2-72 hours after ingestion.

IgE-mediated: immediately - 2hrs following ingestion.

Symptoms:
- Erythematous Rash
- Vomiting
- Diarrhoea ± Constipation
- Blood in Stool
- Mucus in Stool
- Faltering growth

62
Q

Clinical Presentation of Galactosaemia?

A

In-born error of Carbohydrate-metabolism:

GALT - gene mutation

1st few weeks of Life: E.Coli Sepsis
- Vomiting
- Jaundice (conjugated)
- Lethargy
- Hypotonia
- Coagulopathy
- Ascites

Beutler Test: galactose-1-phosphate uridyl transferase.

63
Q

Clinical presentation of MCADD?

A

Disorder mitochondrial fatty acid B-oxidation, due to Medium Chain Acyl Co-A dehydrogenase deficiency. [AR]

Hypoketotic

Hypoglycaemia

Ammonia - elevated

Hypotonia
Vomiting
Jitteriness
Lethargy
Siezures - neuroglycopenia —> Brain Atrophy.
Hepatomegaly Metabolic Crisis

64
Q

Treatment of congenital CMV

A

GANCICLOVIR FOR 6 months

High risk neutropenia

65
Q

Small Pale Nodules Septic Neonate?

A

Listeria monocytogenes

66
Q

Chickenpox with Neurological symptoms
Ataxia + Nystagmus + Headache altered mental state?

A

Acute Cerebelitis

67
Q

Child < 3months

Fever > 38?

A

Perform Full Septic Screen

68
Q

BMI for Obese?

A

> 98th Centile

69
Q

Initial management steps in Nocturnal Enuresis ?

A
  1. Restricted Fluid + Reward System
  2. Alarm system (discontinue dry >2weeks)
  3. Desmopressin (up to 3months)
70
Q

What medication can be used in children with daytime bedwetting?

A

Oxybutynin

71
Q

What is the inheritance of G6PD?

A

X-linked recessive:

Presents with: Prolonged Jaundice or Juvenile -onset

Jaundice
- Pruritis
- Dark Urine

Anaemia
- Pale Skin
- Lethargy

72
Q

What are the clinical features of Kawasaki?

A

5 days Fever

Cervicle Lymphadenopathy > 1.5cm

Maculopapular Morbilliform Rash

Desquamation fingers + toes (> 2wk post fever)

Conjunctivitis B/L non-purulent

Strawberry Tongue + Red Lips

73
Q

Describe the Clinical features of Measles?

A

High Fever

Morbilliform rash - starts behind the ears

Koplik Spots

Conjunctivitis

PHE - notification < 24hrs

74
Q

What is Mobius Syndrome?

A

Facial Nerve Palsy:

B/L Fixed Convergent Squint

No Facial Expressions

‘Drooling’

Cleft Palate

Underdevelopment pectoral muscles

75
Q

What is Brown Syndrome?

A

Vertical Strabismus: Limited elevation of the in adduction.

Restriction of Superior Oblique muscle-tendon-trochlea complex. [IV]

76
Q

Duane Syndrome?

A

Congenital Strabismus - VI Nerve palsy

Limited:

  • adduction
    -abduction
  • retracted eyeball on adduction
77
Q

Eczema Creams?

A

Very Mild - 0.5% Hydrocortisone

Mild - 1% Hydrocortisone

Moderate - Clobetasone (Eumovate)

Severe - Betametasone (Betnovate)

Very Severe - Clobetasol (Dermovate) Specialist

78
Q

Combination of Carbamazepine of Clarithromycin leads to?

A

Increased concentration of Carbamazepine

Clarithromycin - CYP450 inducer

79
Q

Cushing’s Physiological Triad ?

A

Bradycardia

HTN

Respiratory Suppression

[Indicates Raised ICP + impending brain herniation]

Treatment: Immediate Anaesthetic support to secure airway

80
Q

What is the distribution of Epidermolysis Bullosa ?

A

Skin + Mucosal Blistering: Friction points

  • Hands
  • Feet’s
  • Knees
81
Q

What is the distribution of Benign Familial Pemphigus?

A

Symmetrical crusted lesions in flexor surfaces - exacerbated by friction.

82
Q

Management of Hydrocele

A

Spontaneous resolution 18-24 months

  • Surgery is considered > 2yrs
83
Q

What is Leber’s hereditary optic neuropathy?

A

Mitochondrial hereditary disorder

  • Dysfunction of Electron Transport Chain —> Degraded Retinal Cells
84
Q

Paediatric Anti-malarial’s ?

A

Kids > 5kg

Atovaquone

Proguanil

Paediatrics (3 decks)

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