pediatrics

partial seizure

have local onset, involve a small location in the brain

3 types of partial seizures

• simple partial seizures

• complex partial seizures

• partial seizures that generalize

generalized seizures

involve both sides of the brain, no focal onset

• absence seizures

• myoclonic seizures

• atonic seizures

• tonic clonic seizure

focal awareness seizure

• simple partial seizure

• person remains concious, awake and alert

• symptoms mild: tingling, pins and needles, visual / auditory hallucinations, sudden emotion changes

• brief - few seconds to minutes

focal impaired awareness seizure

• complex partial seizure

• conciousness is impaired or altered, person may be dazed or unresponsive

• symptoms: repetitive movements, unusual behaviours, confusion, no awareness of seizure afterwards

• lasts 1-2 mins

absence seizure

• generalized seizure that briefly disrupts conciousness

• person just kind of stares off

• physical symptoms may include rapid eye blinking, face twitching

• typically 5-10 seconds

• after the person is unaware of the seizure and feels normal

myoclonic seizure

• sudden brief shock-like muscle jerks (rapid contraction of muscles)

• usually only last a few seconds

• person is usually conscious and aware of seizure

atonic seizure

• involves sudden loss of muscle tone causing a person to fall or slump

tonic clonic seizure

• involves both a tonic phase (stiffening of the body) and a clonic phase (rhythmic jerking of limbs)

• begins with sudden loss of consciousness, muscles contract causing body to stiffen, body goes into rhythmic jerking movements, generally lasts for 1-2 minutes

postictal phase

after tonic clonic seizure, once jerking movements stop

• confusion, drowsiness, headache, soreness

febrile seizure

seizure associated with fever

• generally occurs with the initial spike in temperature at beginning of illness

• tend to be tonic clonic

status epilepticus

• medical emergency

• tonic clonic seizure that requires medical management because it lasts more than 5 minutes or repeated seizures without the person gaining conciousness between them

management of status epilepticus

• ABCs

• vital signs

• blood glucose

• oxygen (100%)

• iv access

• recovery position

• meds: lorazepam, keppra/levetiracetam

management of chronic seizures

• drug therapy: keppra, valproic acid, etc.

• ketogenic diet: high fat, low carb, mod protein (induces ketosis, makes primary fuel source ketones).

• vagus nerve stimulation

• surgical therapy (temporal lobectomy, hemispherectomy)

hemophilia

• rare inherited blood disorder that causes blood to clot less

• happens when the body doesn’t make enough clotting factors

hemophilia A

• most common type

• happens when the person does not have enough clotting factor VIII (8)

hemophilia B

happens when the person does not have enough clotting factor IX (9)

diagnosis of hemophilia

• family hx

• factor levels

• PTT

treatment for factor VIII deficiency

may give DDAVP (desmopressin)

• synthetic hormone that stimulates the release of stored factor VIII from the endothelial cells lining blood vessels

• only lasts 6-8 hours, used for short term management for surgeries, dental procedures, etc.

how is fetal circulation different from adult circulation?

• oxygenation is provided by the placenta.

• the foramen ovale, an opening between the two atria allow blood to flow from the right to left atrium.

• the ductus arteriosus allows blood to flow from the pulmonary artery to the aorta.

• these shunts should close when the baby takes its first breath.

types of congentital heart defects (4)

• CHD that increase pulmonary blood flow

• CHD that decrease pulmonary blood flow

• obstructive defects

• mixed defects

CHD that increase pulmonary blood flow (acyanotic)

increased pulmonary bloodflow increases work of the heart

• patent ductus arteriosus (PDA)

• ventricular septal defect (VSD)

• atrial septal defect (ASD)

patent ductus arteriosus (PDA)

• shunt does not close

• connection between the aorta and the pulmonary artery

what can be given to infants to stimulate the closure of the ductus arteriosis?

NSAIDs!

what are kids at risk for with a patent ductus arteriosis?

• frequent infections

• fatigue

• poor growth

• harsh murmur

ventricular septal defect (VSD)

• hole between right and left ventricle

• can increase the amount of blood that goes back to the lungs

• can cause pulmonary hypertension

atrial septal defect (ASD)

• hole between right and left atria.

• 80% will close spontaneously, if not closed by age 3 will need surgical closure.

CHD that decrease pulmonary blood flow (cyanotic)

there is an obstruction of blood flow to lungs, pressure in the right side becomes greater than left. deoxygenated blood bypasses lungs and mixes with oxygenated blood to go into circulatory system

• tetralogy of fallot

• tricuspid atresia

how do the kidneys compensate for cyanotic CHD?

• the kidneys produce more erythropoietin to stimulate bone marrow to produce mroe RBCs

• leads to polycythemia - blood becomes more viscous, increases work of the heart, more at risk for clots

tetralogy of fallot

four defects:

• pulmonary stenosis - decreased blood flow to the lungs.

• ventricular septal defect (VSD) - connection b/w right and left ventricle.

• overriding aorta - aorta is positioned over both ventricles, getting mixed blood into circulation.

• right ventricular hypertrophy - right ventricle enlarged due to pulmonary stenosis (RV has to work harder to push blood past pulmonary valve).

TET spells + treatment

periods of hyper cyanosis, happens in kids diagnosed with tetralogy of fallot

• cyanotic, hypoxemia, dyspnea, agitation.

• often happens during periods of crying or feeding.

treatment:

• oxygen

• knee to chest position

• morphine (0.1mg/kg)

• propanolol (relaxes vessels)

tricuspid atresia

• tricuspid valve fails to develop, blood cannot flow from right atrium to right ventricle.

• blood will pass through to the left atrium via the ASD, never ending up in the pulmonary artery to get oxygenated.

• blood travels to lungs through the PDA.

obstructive defects

some sort of narrowing of a major vessel interfering with the blood flow through the vessel.

• coarctation of the aorta

• aortic stenosis

coarctation of the aorta

narrowing of the aorta

• bp increased in the heart and upper portions of the body and decreased in lower parts of the body.

• can lead to HF.

• collateral circulation can develop as the body attempts to maintain bloodflow to the descending aorta.

• risk for aortic rupture, aneurysm, and stroke.

s/s of coarctation of the aorta

• leg pain with activity

• dizziness / fainting

• headache

• bounding pulses in upper extremities

• weak pulses in lower extremities

• blood pressure 20mmhg higher in upper extremities than lower extremities

aortic stenosis

• obstruction of the valve itself or obstruction just above the aortic valve.

• left ventricle has to work harder to pump into the aorta.

• decreased cardiac output, backflow of blood, pumonary edema, HF.

• generally asymptomatic. may hear murmur at left sternal border.

mixed defects

• transposition of the great arteries

transposition of the great arteries (TGA)

pulmonary artery and the aorta are flipped (transposed)

• blood flows in a circle never getting oxygenated.

• deoxygenated blood keeps getting pumped back to body without getting to lungs.

• oxygenated lungs keep going back to lungs in a loop.

treatment of transposition of the great arteries

continuous infusion of prostoglandins to keep PDA open so some blood is able to get to lungs

hypoplastic left heart syndrome (HLHS)

all the structures on the left side of the heart are underdeveloped

• mitral and aortic valves are either completely closed or tiny.

• the LV is not functioning.

• right side of heart is only functioning part, having to pump blood to the lungs and to the rest of the body. when the PDA closes in the next few days, the heart cannot pump blood to the systemic circulation. = poor perfusion + shock.

• only treatment is heart transplant. may use inotropes to increase strength of heart, furosemide to get rid of extra fluid.

hypoplastic left heart syndrome (HLHS) symptoms

• tachycardia

• hypothermia

• increased work of breathing

• pallor of the extremities

• decreased SPO2 (in 70s) - normally don’t give them O2

• poor weight gain (hard time feeding)