FOP Flashcards
(84 cards)
What feature is mostly suggestive of “Orbial Cellulits”
Restricted Eye Movements:
suggests involvement of the orbital septum indicating deeper involvement.
Tx : IV-antibiotics
Imaging: CT / MRI Head
Refer:
-> Ophthalmology
-> ENT
S/E: Medications in Breast Milk:
1) Tetracycline
2) Isoniazid
3) Chloramphenicol
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)
TRUE OR FALSE:
Examination of the Throat is essential in Children that present with Stridor ?
FALSE:
Throat examination should be avoided in children presenting with Stridor, as this can provoke or worsen upper airway obstruction.
Signs of Foreign-body inhalation ?
a) Clinical
b) CXR
ASYMMETRY
a) Asymmetrical Chest Expansion or Wheeze
b) Asymmetrical Hyper-expansion of Lung Fields
What is Kartagener Syndrome ?
Subset:
Primary Ciliary Dyskinesia
Characteristic Triad:
1) Bronchiectasis
2) Chronic Sinusitis
3) Situs Inversus
What are the Stages of Clubbing ?
1) Fluctuant Nail-bed
SIDS Advice:
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
Cholera Vaccination Regime [NICE] ?
2-6yrs: 3 doses [1-6 wks apart]
6-17yrs : 2 doses [1-6 wks apart]
What type of Vaccine is the Cholera Vaccine?
Dukoral - inactivated oral vaccine
(rCTB) - recombinant cholera toxin B
4 types of Killed V.Cholerae
1st Line Invx for Hypertension in Kids?
Ambulatory BP-monitoring (ABPM)
What are the characteristic features of Noonan’s Syndrome:
“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
Inheritance of Noonan’s syndrome?
Autosomal-DOMINANT
How does Cystinosis present?
Cystinosis: Autosomal Recessive [AR]
Lysosomal recessive disease
- Nephropathy
- Poor Growth
- Hypophosphataemic Rickets
What is the characteristic triad for hypospadius?
1) Foreshortened Ventral opening
2) Chordee [Ventral Tilt]
3) Hooded foreskin
Neonate with Double Bubble Sign on AXR:
Duodenal Atresia:
- More prevalent in T21 [Down’s Syndrome]
- Bilious Vomiting from 1st feed (< 24hrs)
How does SMA - Type 1 present at brith?
“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
Day 5 Heel Prick:
1) Glutaric acid Uris type I
2) Isovaleric acidaemia
3) MCADD
4) Phenylketonuria
5) Cystic Fibrosis
6) Congenital Hypothyroidism
What is VACTERAL?
Vertebral Anomalies
Anorectal malformation
Cardiac defects
Tracheo-oesophageal fistulae
Renal anomalies
Limb deformities
Anorectal - Urethral Fistualae with Anorectal malformation
1) De-functoning colostomy
2) Micturating cyst-urogram
3) Primary anoplasty
Grades of IVH:
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]
SMA-Type 1:
Autosomal Recessive [AR]
Deletion of SMN-gene Ch 5q
Severe form presents in the neonatal period with:
- Severe Hypotonia
- Feeding difficulties
- Respiratory distress
Ladds Procedure:
Correction of Malrotation - widening of the mesenteric base
What is Choanal Atresia?
Congenital blockage of the Nasal passages, which can occur unilaterally or bi-laterally
- Unilateral: Recurrent Nasal Drainage,
Recurrent Sinus infections
- Bilateral: Resp.Distress + Cyanosis
Congenital Myotonic Dystrophy [DM1]
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)