Peads 17th Nov Flashcards

1
Q

What is a generalised seizure?
Absence? Clonic? Tonic? Tonic-clonic? Myoclonic? Atonic?
When would you be worried about status eplilepticus ?

A

Generalised: Involve both hemispheres – Includes ABSENCE, CLONIC, TONIC, TONIC-CLONIC, MYOCLONIC and ATONIC seizures
Absence – frequent and brief. Unresponsive/look blank/stare or eyelid flutter. Often unnoticed. Respond well to anti-epileptics. Most grow out of it
Clonic – rare, repeated rhythmic jerky movements of limbs, not stoppable by restraining them.
Tonic – arm/s or leg/s suddenly stiffen forcefully. Last <20 seconds. Consciousness preserved, though often occur during sleep
Tonic-clonic – Muscles stiffen – causing cry/groan, then loss of consciousness, followed by “clonic” jerking movements. Often tongue biting, and loss of bladder/bowel control. Last 1-3 mins. Longer than this = Status epilepticus (technically 30 mins but you’d intervene long before this if suspected!) groggy and disorientated after the event
Myoclonic – Brief shock-like jerks of muscle/group of muscles. Many different characteristics – patient awake and clear-thinking
Atonic – abrupt loss of muscle tone – sudden floppiness. A.k.a “drop attacks”

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2
Q

What is the cause of non epileptic attacks ? Usual trigger? What might you want to take from hx? Do they need antiepileptics? Difference between an long NEA and status epliepticus?

A

Psychological triggers - usually strong emotion
Find out about other psychological disorders
No need for drugs

tatus epilepticus, though it is continuous fitting for ~30 mins, still occurs in a stop-start nature for that time, in NEA, the jerks are non-stop at all for that whole time (subtle and hard to spot though!)

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3
Q

Sx of L->R shunt? Egs ?

A

Breathless (may be aSx) - a cyanotic

ASD, VSD, PDA

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4
Q

Sx of r->L shunt ? Egs ?

A

Blue, breathless, cyanotic

ToF
TGA

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5
Q

Sx of common mixing? Egs?

A

Cyanotic, breathless and blue

AVSD, complex disease

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6
Q

Sx of well children with obstruction? Egs?

A

ASx

AS, PS, adult type coarctation of aorta

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7
Q

Sick neonate with obstruction sx? Egs?

A

A cyanotic - collapsed with shock
Coarctation
Hypoplastic left heart syndrome

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8
Q
Buzzwords 
Projectile vomiting?
Machine like murmur under left clavicle?
Blue sclera?
Red currant jelly stool?
Infant (unable to walk) with femur fracture? 
White stool?
“Walking up” legs to hands?
A

Buzzwords
Projectile vomiting - pyloric stenosis (also get olive size/shape lump at pyloric region)

Machine like murmur under left clavicle? - PDA
Blue sclera? - osteogenesis imperfecta
Red currant jelly stool? - intussusseption
Infant (unable to walk) with femur fracture? Non accidental
White stool? - binary atresia
“Walking up” legs to hands? - duchenne muscular dystrophy

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9
Q

No obvious cause of fever in child - what to investigate?

A

Skin – expose child, check for rash (cellulitis e.g.)
ENT exam – otitis media, tonsillitis (throat swab)
Urinary system – Clean catch urine sample or voluntary urine sample from older child for dipstick and MSU cultures
Gastro – ask about what they’ve eaten, stool sample
Respiratory infection – listen to chest, CXR if suspected

Most very young children will just be off-mood as an initial presentation, ALWAYS listen to mum (or dad of course) if they say their kid doesn’t seem quite right, and investigate everything to locate the source of febrility/infection!

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10
Q

When do kids normally get reactive arthritis ? What syndrome has arthritis and two other sx? Mx?

A

ollows enteritis, but sometimes post-chlamydia or rheumatic fever. In Reiter’s Syndrome, it occurs with Urethritis and Conjunctivitis (“Can’t see, can’t pee, can’t climb a tree!”) – steroid injections and NSAIDS

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11
Q

When do you diagnose juvenile idiopathic arthritis? Who is it more common in? Mx?

A

Diagnosed if arthritic for >6 weeks once other causes excluded. More common in girls.
Physiotherapy
medications (NSAIDS, systemic steroids in severe cases and Methotrexate)

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12
Q

What is still’s disease? Who does it affect? Where? Other sx?

A

young kids, primarily affects knees, wrists, and ankles. Accompanies systemic illness – fever, rash, hepatosplenomegaly, pericarditis

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13
Q

What is rheumatoid factor?

A

Proteins that produced by immune system and attack healthy tissue in the body

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14
Q

Polyarticular arthritis. Which joints not affected? May get systemic what?

A

Any joints bar MCPs

Systemic vasculitis which is associated with poor functional outcome

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15
Q

Who gets oligoarticular arthritis? Pattern of affect? What is present? What may some people develop?

A
Most common of the childhood arthritis 
Young girls 
Asymmetrical 
Antinuclear antibodies are present 
some may get chronic uveitis
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16
Q

2 things you need to rule out with any child with limp?

A

Septic arthritis

Osteomyelitis

17
Q

What is perthe’s? Presentation? What does it lead to? What happens on recovery and how long does it take? Who is it more common in? Diagnosis and prognosis?

A

Idiopathic / avascular necrosis of head of femur

Insidious limp onset around 6yrs with intermittent pain and limited movement

Leads to growth disturbance -> once growth resumes the area revascularises and bone reossifies (takes ~3years)

Boys
X-ray - prognosis is better with earlier age of onset

18
Q

Prognosis in transient synovitis ? Presentation? How to diagnose / what do you need to do? Mx?

A

Self limiting in 2/52
Sudden onset hip pain - refusal to weight bear, limp
Diagnosis of exclusion - rule out septic arthritis (afebrile and no pain at rest) - if suspected septic hip -> start Abx and aspirate
Pain relief and rest

19
Q

How common is slipped upper/capital femoral epiphysis? What happens and when? Common in? Presentation? Complication? Mx?

A

Rare
Femoral head slips on femoral neck through epiphysis during pubertal growth spurt
Boys and usually overweight (weight often in exam Q)

Limp and hip pain that may radiate to knee
May -> AVN of femoral head
Surgical correction

20
Q

What is osgood schlatter? Who gets it when?

A

Common, benign, self limiting knee pain
Inflamed periosteum on tibial tuberosity causing tenderness
Traumatic cause in VERY ACTIVE teens

21
Q

4 conditions in paeds usual GI surgery ?

A

Intussusseption
Hirchsprungs
Pyloric stenosis
Oesophageal atresia

22
Q

What is intussusseption? Where does it normally occur? Presentation ? O/e? What can happen? Usual age? Diagnosis? Mx?

A

Telescoping of one section of the bowl into another usually at ileocecal valve
Vomiting and episodic severe colicky abdo pain
Sausage shaped mass in RUQ - red currant stools
Develop obstruction and shock
6 months most common
USS and barium enema to diagnose
Correct with air insufflation enema
-> if unsuccessful / signs of perforation -> operative reduction

23
Q

What causes hirschprungs? More common in? Common presentation? Diagnosis? Mx?

A

Lack of ganglion migration during development to section of bowel – this segment is narrow and contracted.

More common in boys, often family history.

Presents (usually) neonatally with failure to pass meconium, followed by bilious vomiting.
Diagnose with suction rectal biopsy (lack of ganglion cells).

Correct with Duhamel pull-through operation (anastomose normal bowel to anus, resect aganglionic bowel)

24
Q

Pyloric stenosis presentation? O/e? Usual age? Mx?

A

Projectile vomiting at age ~6weeks
Olive shaped lump at pylorus
Operative correction - pyloric myotomy

25
Q

Presentation of oesophageal atresia? Risk? Diagnosis?

A

Immediat vomit / possessing after feed -> risk of aspiration
Pass nasogastric tube and will see curl back on itself on X-ray

26
Q

Newborn screening - what inherited metabolic conditions do you test for?

A

Heel-prick/bloodspot test: Checks for presence of any of: Sickle Cell, Cystic Fibrosis, Congenital Hypothyroidism, and one of 6 inherited metabolic conditions:
phenylketonuria (PKU)
medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
maple syrup urine disease (MSUD)
isovaleric acidaemia (IVA)
glutaric aciduria type 1 (GA1)
homocystinuria (pyridoxine unresponsive) (HCU)

Hearing test to check for congenital deafness
Eyes - appearance and movement to check for cataracts
Heart – pulses and auscultation – catch any murmurs or coarctation
Hips – Ortolani method, pushing hips at 90 degrees aainst your own hands to see if they dislocate, then fully abduct the hips to see if they then relocate – this is to exclude Developmental Dysplasia of the Hip (DDH)
Genitals – look for any ambiguous genitalia (?CAH) and make sure both testicles have descended, make sure anus isn’t imperforate

27
Q

What is alports syndrome? Which organs does it affect?

A

Collagen IV deficiency / absence

Glomerulus of kidney
Eye
Cochlea

28
Q

Sx of alports?

A

Kidneys function normal in early childhood
Over time non functioning collagen -> pour us GBM
-> microscopic haematuria -> gross haematuria + proteinuria

Sclerosis of GBM

-> renal failure -> renal vascular hypertension

EAR
Hearing loss [sensoryneural] that starts in childhood / adolescence

EYE
Anterior lenticonus (lens capsule not strong enough)
-> myopia and lens opacity

29
Q

Usual cause of alports?

A

Mutation of COL4A5 (collagen IV protein A5) on X chromosome

30
Q

Diagnosis of alports?

A

Skin/kidney biopsy - imunoflorescence
->degraded collagen not detected

Electron microscopy - GBM is thick and abnormal

Gene testing

31
Q

Treatment of alports

A

Proteinuria -> ARBs / ACEi

Anterior lenticonus -> lens transplant

Kidney failure -> dialysis / transplant

32
Q

JIA acronym

A

FLAAAP

Fever

Arthralgia
Anorexia
Anterior Uveitis

Pink (salmon) rash