FOP Flashcards

(84 cards)

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

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

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

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

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

What is Kartagener Syndrome ?

A

Subset:
Primary Ciliary Dyskinesia

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

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

What are the Stages of Clubbing ?

A

1) Fluctuant Nail-bed

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

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

Cholera Vaccination Regime [NICE] ?

A

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

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

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

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

1st Line Invx for Hypertension in Kids?

A

Ambulatory BP-monitoring (ABPM)

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

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

Inheritance of Noonan’s syndrome?

A

Autosomal-DOMINANT

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

How does Cystinosis present?

A

Cystinosis: Autosomal Recessive [AR]
Lysosomal recessive disease

  1. Nephropathy
  2. Poor Growth
  3. Hypophosphataemic Rickets
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the characteristic triad for hypospadius?

A

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

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

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

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

What is VACTERAL?

A

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

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

Anorectal - Urethral Fistualae with Anorectal malformation

A

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

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

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

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

Ladds Procedure:

A

Correction of Malrotation - widening of the mesenteric base

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

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Haematology Bloods in DIC:
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
26
Haematology bloods in Haemolytic Newborn Disease [HND]:
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]
27
Haematology bloods in Haemophilias ?
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
28
At what gestation does Surfactant get produced ?
24 - 28 wks
29
In what conditions can Exopthalmos Present ? [4]
BWS - Beckwith-Wiedemann Syndrome T21 T18 Pentalogy of Cantrell
30
Clinical Features: [5] Beckwith-Wiedemann Syndrome [BWS]
Rare - genetic condition 1) Exomphalos 2) Macroglosia 3) Visceromegaly 4) Pancreatic hyperplasia - > (H) Insulin production -> recurrent hypoglycaemia 5) Genitourinary abnormalities
31
What disease is the biggest risk factor for fungal pneumonia?
Chronic granulomatous disease; X-linked recessive (CYBB gene) Inability of phagocytes to kill and engulf organisms due to defective enzymes.
32
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
Juvenile Angiofibroma
33
What are the characteristics features of Tuberous Sclerosis Complex?
[AD] multisystem disease - Hamartomas - Brain —> Seizures [80%] Infantile Spasms [50%] - Heart - Lungs - Skin
34
What is Precocious Puberty?
Early pubertal development: Girls < 8yrs (commonly idiopathic) Boys < 9yrs (commonly pathological)
35
What is the average age of Breast development “Thelarche” in girls?
11 years (Absence by 13yrs is considered delayed)
36
What are the 2 main causes of Acquired Hypothyroidism?
1. Autoimmune (Hashimoto’s) Thyroiditis 2. Post-Total-Body-Irradiation (TBI) - e.g. post bone-marrow biopsy
37
Klinefelter Syndrome, disease associations?
Pulmonary Disease Varicose Veins Breast Cancer Leukaemias Mediastinal germ-cell tumours
38
What is the Prevalence of Delayed Puberty ?
More common in boys, usually idiopathic
39
What are the affected organs in multiple endocrine neoplasia [MEN1]
Pancreatic Insulinoma Pituitary Tumour Parathyroid Hyperplasia —> Parathyroidism - (H) Ca2+ increased bone mobilisation - (L) PO4- increased renal excretion FHx: Maternal Prolactinoma
40
What structure is most likely to be affected by suprasellar extension of a Pituitary Adenoma?
Optic Nerve (Optic Chiasm) —> Bitemporal Hemianopia
41
What is McCune-Albright Syndrome?
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
What is the most common cause of Cardiac defects in Children?
VSD * Alchohol increases risk of septal defects *
43
CXR sign “egg on string”, with Cyanosis with reduced pre+post ductal sats?
TGA
44
Able to roll, babbling, reaching for objects, sit with support?
6 months
45
Able to roll, not reach for objects
4 months
46
Sit-unsupported
9 months
47
2 word sentences Use a fork Stack x6 blocks
2 yrs-old
48
3 word sentences Know own name ‘Tri’-cycle
3 yrs
49
Balance on 1 foot Brush his teeth independently Write his name No nappies (dry day & night)
5 yrs-old
50
Use scissors & buttons Not dry @ night Not be able to spell name
4 years
51
Lyme disease treatment
Amoxicillin
52
< 3months old Bacterial meningitis management?
IV-Cefotaxime (Ceftriaxone can exacerbate hypebilirubinaemia in <3 months —> Kernicterus)
53
Sepsis management?
Cefotaxime/Ceftriaxone(hyperbilirubinaemia risk) Amoxicillin / Ampicillin < 28 days Gentamicin [Severe] ± Aciclovir
54
What are the features of congenital Parvovirus (B19) ?
Non-immune Hydrops Fetalis Miscarriage (9-20wks) Severe Anaemia Cardiac Failure (Viral Myocarditis) Non-blanching rash Extramedullary Haematopoesis
55
Signs of Congenital CMV? [5]
IUGR Microcephaly Petichial/purpuric rash Hepatosplenomegaly Peri-ventricular calcification Cranial - ventriculomegaly Chorioretinitis / Cataracts Sensorineural Deafness
56
Signs of Congenital HSV [3]?
Microcephaly Cutaneous Scars Vesicles
57
Signs of Congenital Rubella? [6]
Cataracts Cardiac Defects: - PDA - Pulmonary Artery Stenosis B/L SNHL Hepatosplenomegaly Microcephaly Petechiae
58
Signs of Congenital Syphilis? (8)
Coryzal Hepatosplenomegaly Maculopapular rash - palms + soles Hydrops Vesicles Condylomata Lata Bone Abnormalities Pseudoparalysis
59
Signs of Congenital Toxoplasmosis? [4]
IUGR Hydrocephalus Intercerebral calcification Chorioretinitis
60
Signs of Congenital Zika Virus? [5]
1. Microcephaly 2. Intracranial calcification 3. Ocular abnormalities 4. Hypertonia 5. Joint Contractures [Arthrogyposis]
61
Sign’s Cow’s milk protein allergy ?
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
Clinical Presentation of Galactosaemia?
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
Clinical presentation of MCADD?
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
Treatment of congenital CMV
GANCICLOVIR FOR 6 months High risk neutropenia
65
Small Pale Nodules Septic Neonate?
Listeria monocytogenes
66
Chickenpox with Neurological symptoms Ataxia + Nystagmus + Headache altered mental state?
Acute Cerebelitis
67
Child < 3months Fever > 38?
Perform Full Septic Screen
68
BMI for Obese?
> 98th Centile
69
Initial management steps in Nocturnal Enuresis ?
1. Restricted Fluid + Reward System 2. Alarm system (discontinue dry >2weeks) 3. Desmopressin (up to 3months)
70
What medication can be used in children with daytime bedwetting?
Oxybutynin
71
What is the inheritance of G6PD?
X-linked recessive: Presents with: Prolonged Jaundice or Juvenile -onset Jaundice - Pruritis - Dark Urine Anaemia - Pale Skin - Lethargy
72
What are the clinical features of Kawasaki?
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
Describe the Clinical features of Measles?
High Fever Morbilliform rash - starts behind the ears Koplik Spots Conjunctivitis PHE - notification < 24hrs
74
What is Mobius Syndrome?
Facial Nerve Palsy: B/L Fixed Convergent Squint No Facial Expressions ‘Drooling’ Cleft Palate Underdevelopment pectoral muscles
75
What is Brown Syndrome?
Vertical Strabismus: Limited elevation of the in adduction. Restriction of Superior Oblique muscle-tendon-trochlea complex. [IV]
76
Duane Syndrome?
Congenital Strabismus - VI Nerve palsy Limited: - adduction -abduction - retracted eyeball on adduction
77
Eczema Creams?
Very Mild - 0.5% Hydrocortisone Mild - 1% Hydrocortisone Moderate - Clobetasone (Eumovate) Severe - Betametasone (Betnovate) Very Severe - Clobetasol (Dermovate) Specialist
78
Combination of Carbamazepine of Clarithromycin leads to?
Increased concentration of Carbamazepine Clarithromycin - CYP450 inducer
79
Cushing’s Physiological Triad ?
Bradycardia HTN Respiratory Suppression [Indicates Raised ICP + impending brain herniation] Treatment: Immediate Anaesthetic support to secure airway
80
What is the distribution of Epidermolysis Bullosa ?
Skin + Mucosal Blistering: Friction points - Hands - Feet’s - Knees
81
What is the distribution of Benign Familial Pemphigus?
Symmetrical crusted lesions in flexor surfaces - exacerbated by friction.
82
Management of Hydrocele
Spontaneous resolution 18-24 months - Surgery is considered > 2yrs
83
What is Leber’s hereditary optic neuropathy?
Mitochondrial hereditary disorder - Dysfunction of Electron Transport Chain —> Degraded Retinal Cells
84
Paediatric Anti-malarial’s ?
Kids > 5kg Atovaquone Proguanil