Peds Conditions Barry
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| 2 factors cause intraventricular hemorrhage: | 1. Loss of cerebral autoregulation
2. Abrupt alterations in cerebral blood flow and pressures
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| When is the highest occurrence of intraventricular hemorrhage? | Highest occurrence in first 72 hours of life.
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| Increased incidence of intraventricular hemorrhage in what population? | Incidence up in infants < 32 weeks gestation and with birth weight < 1500 grams.
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| To prevent intraventricular hemorrhage, avoid conditions that: | -Interfere with autoregulation (hypocarbia, hypercarbia,hypoxia, acidosis)
-Overwhelm autoregulation abilities (Hypertension)
-Contribute to rapid fluctuations of cerebral blood flow or pressure
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| Primary cause of respiratory distress syndrome (RDS): | Primary Cause: surfactant deficiency secondary to immature type II alveolar cells in the preterm lung.
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| When does surfactant appear, when is it mature? | Surfactant appears in amniotic fluid 28-32 weeks and mature levels by 34-35 weeks.
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| What can cause earlier release of surfactant? | Maternally administered steroids.
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| Tell me the story of what happens after hypoxia in the infant (RDS)? | Increase work of breathing-> hypercarbia-> pulmonary arterial vasoconstriction->R to L shunting and ischemic injury to cells producing surfactant.
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| On an RDS Xray you will see: | Bell shaped thorax with diffuse symmetrical ground glass infiltrates.
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| Treatment for RDS: | -Supplemental Oxygen for target -PaO2 of 55 – 70 mm Hg
-CPAP up to 10 cmH2O
-Mechanical ventilation
-Avoid oxygen toxicity and pulmonary barotrauma
-Artificial surfactant
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| PDA = Failed closure or reopening after functional closure. What happens? | -Left to right shunt occurs and may be asymptomatic or result in CHF and failure to thrive if large
-If PVR remains high the R to L shunt results in hypoxemia
-Obligatory PDA occurs in 10% of infants with CHD
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| Physical signs of R to L shunt: | -Hyperdynamic precordium
-Wide pulse pressures
-Bounding arterial pulses
-Increased oxygen requirements
-Metabolic acidosis
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| Diagnosis or R to L shunt is by: | ECHO.
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| Treatment for PDA: | Indomethacin
Fluid restriction
Diuretics for CHF Surgery
Mechanical ventilation
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| Hypoglycemia in a neonate = | less than 40
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| Hyperglycemia in term and preterm infants: | over 125 and over 150
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| To maintain normoglycemia, what is required? | Neonates require 3-5 mg/kg per minute and preterm neonates 5-6 mg/kg per minute of glucose to maintain euglycemia.
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| What are 4 neonate thermoregulatory disadvantages? | -Increased surface are relative to metabolic mass
-Smaller heat sink reserve
-Less insulating fat
-Decreased skin keratinization = more evaporative heat loss
-Inability to shiver
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| To create heat, neonates rely on 2 metabolically costly mechanisms: | -Brown fat metabolism
-Shunting of hepatocyte oxidative phosphorylation to the thermogenic proton leak pathway
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| Neonates creating heat results in what bad stuff? | Acidosis, hypoxia, hypoventilation and hypotension
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| Postoperative apnea - who is at greatest risk? | Infants 55weeks of post-conceptual age & younger are at greatest risks.
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| Retinopathy of prematurity - who is at greatest risk and why? | PRETERM INFANTS <1500 G AND 34 WEEKS GESTATION @RISK - RETINAL VASCULATURE NOT FULLY MATURE.
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| What happens in retinopathy of prematurity - in caps, please? | VESSELS CAN VASOCONSTRICT, HEMORRHAGE, AND VASOPROLIFERATE IN RESPONSE TO HYPEROXIA.
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| When is vascularization of the eye complete? | 44 weeks.
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| Blindness can result from retinal detatchment - what has been implicated in causing retinopathy of prematurity? | HYPOXEMIA, ACIDOSIS, HYPOTENSION, AND SEPSIS HAVE BEEN IMPLICATED IN CAUSING ROP.
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| You should measure O2 where? | Preductal (Rt hand or ear).
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| To avoid ROP, where should you keep your O2 during GETA? | 93-96%
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| How much type 1 fibers in diaphragm? | 10-25%
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| What is most common laryngeal disorder of newborn? | Laryngomalacia.
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| What is laryngomalacia? | Inward collapse of epiglottis and aryepiglottic folds during inspiration.
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| What are signs of laryngomalacia? | Stridor especially when infant lying supine, crying, or feeding.
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| What other condition is associated with laryngomalacia? | Possible association of gastroesophageal reflux.
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| Can you easily PPV a child with laryngomalacia? | Yes.
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| Is laryngomalacia relieved with an oral airway? | No.
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| What's associated with OSA? | -Habitual snoring, daytime somnolence, behavioral problems, school problems, developmental delay
-Failure to thrive
-Hypoxemia, hypoventilation, PHTN, CHF and death
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| Can OSA cause Cor Pulmonale? | Hell yeah it can.
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| Many patients with craniofacial abnormalities can also have: | OSA.
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| What is the most common acquired anomaly of the larynx in children and most common cause of tracheostomy for children younger than 12 months? | Subglottic Stenosis
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| What sadly causes subglottic stenosis? | Chronic inflammation and scarring from the presence of an endotracheal tube.
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| Is it easy to PPV a patient with subglottic stenosis? | PPV may be difficult.
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| Tracheomalacia is: | Defined as a softening of the tracheal cartilage.
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| Tracheomalacia causes airway collapse during: | Forceful coughing or exhalation ventilation.
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| Patients with tracheomalacia have these symptoms: | Long term mechanical ventilation, noisy breathing, “barky” cough, wheezing and respiratory distress.
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| Asthma is a chronic disease of reversible airway obstruction and characterized by: | Bronchial hyperreactivity, inflammation, & mucous secretion.
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| Asthma pharmacotherapy: | -Beta adrenergic agonists
-Corticosteroids
-Cromolyn sodium
-Anticholinergics
-Leukotriene pathway modifiers
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| Unfortunately, for intraoperative bronchospasm, Intraoperative bronchospasm efficiency of MDI of albuterol through 3.0 – 6.0 mm ID tracheal tubes is: | Low.
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| What is cystic fibrosis? | A hereditary disease of exocrine glands primarily affecting the pulmonary and GI systems.
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| Cystic fibrosis causes exocrine pancreatic... | Insufficiency.
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| Cystic fibrosis causes GI... | Obstruction.
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| In a patient with cystic fibrosis, how does sweat change? | They lose an excess of sodium and chloride.
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| Sinopulmonary disease is found in... | Cystic fibrosis patients.
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| Cystic fibrosis patients' sbnormal thick and viscous secretions coupled with decreased ciliary activity lead to... | Pneumonia, wheezing and bronchiectasis
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| What does cystic fibrosis do to pulmonary function? | Increases residual volume and airway resistance with decreased vital capacity & expiratory flow rate.
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| Cystic fibrosis may lead to malabsorption syndrome which may lead to... | Malabsorption syndrome may lead to dehydration, electrolyte disorders and Vitamin K deficiencies (Clotting factors).
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| Cystic fibrosis patients have a higher incidence of this cardiac condition: | Right ventricular hypertrophy.
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| Common surgical procedures for the patient with cystic fibrosis: | -Endoscopic sinus surgery (Nasal Polyps)
-CVP catheter placement
-Bronchoscopy
-Lobectomy
-Pneumonectomy
-Abdominal exploration
-Colostomy
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| N2O in a patient with cystic fibrosis? | NO! Avoid N2O; increase FiO2; Inhalational uptake prolonged. Rapid emergence good.
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| Do cystic fibrosis patients have issues with hyperactive airway? | Uh, yeah.
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| In a patient with meconium aspiration, what is believed to be responsible for the violent inflammatory response? | Bile salts.
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| Can pulmonary hemorrhage occur in meconium aspiration? | Yes.
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| Therapy for patients with meconium aspiration | -Amnioinfusion
-Tracheal Intubation and suctioning
-Antibiotics
-Surfactant Replacement
-ECMO for severe MAS with PPHN
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| Bronchopulmonary dysplasia is: | BPD is a chronic disease of lung parenchyma and small airways with chronic respiratory insufficiency in prematurely born infants.
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| Bronchopulmonary dysplasia is oxygen dependence at when? | BPD is oxygen dependence at 36 weeks postconceptual age.
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| What is cerebral palsy? | It is an abnormality of posture and movement caused by brain injury occurring before 2 years of age.
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| What causes cerebral palsy? | Prematurity, LBW, prenatal infections (rubella), congenital malformations of the brain, meningitis, brain tumors, and intracranial hemorrhage.
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| Describe pyramidal type of cerebral palsy: | Pyramidal type: damage to cerebral cortex usually produces spasticity, contractures and bony deformities.
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| Describe extra-pyramidal type of cerebral palsy: | Extra-pyramidal type: damage to the basal ganglia or cerebellum tens to produce athetosis or ataxia.
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| Can pyramidal and extra-pyramidal type of cerebral palsy co-exist? | Yes, and do, often.
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| What is baclofen? | Baclofen is a GABA receptor agonist with action in the dorsal horn of the spinal cord. It is used to reduce pain with muscle spasms and delay development of contractures. Abrupt withdrawal issues.
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| What does botox do/anesthesia issues? | Botox produces reversible muscle denervation & temporary reduction in muscle tone. No anesthesia issues.
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| Why do patients with cerebral palsy have hypothermia issues? | Hypothalamus issues.
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| Do CP patients have latex allergy issues? | Yes.
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| What is the inherited, sex-linked recessive disease that presents in early childhood between 2 and 6 years of age? | Duchennes's Muscular Dystrophy.
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| T or F: Duchenne's patients look like they're wasting away? | F: they LOOK very muscular.
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| S/S of Duchenne's: | -Symmetric skeletal muscle wasting
-Wasting of pelvics – Gower’s sign
Hypertrophy of skeletal muscles
Kyphoscoliosis
EKG changes: tall R in V1, deep Q in limb leads, short P-R interval, tachycardia
Mitral valve prolapse
Intestinal hypomobility and d
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| Sux and muscular dystrophy? | Hyperkalemia
Massive rhabdomyolysis
Cardiac arrest. Good God no don't do it!
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| Non-depolarizing muscle relaxants and MD: | OK: prolonged recovery time.
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| What can't you give to patients with congenital myotonic dystrophy? | No succinylcholine – induces intense generalized myotonic contraction.
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| Heart and congenital myotonic dystrophy? | Fifty to ninety % have myocardial conduction defects, bradycardia and 1st degree AV block are common.
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| In patients with congenital myotonic dystrophy, reversal? | Reversal of muscle relaxation with anticholinesterase drugs may precipitate or enhance myotonia.
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| Osteogenesis imperfecta: | Congenital connective tissue disorder that causes a generalized decrease in bone mass and bones that break easily.
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| Clinical manifestations of osteogenesis imperfecta: | Blue sclera, dental abnormalities and brittle bones. Deafness, abnormal platelet function, increased BMR & O2 consumption, cleft palate, vascular fragility, hydrocephalus, spina bifida and aortic and mitral regurgitation.
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| Scoliosis and osteogenesis imperfecta? | About 20% of the time.
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| Sux and osteogenesis imperfecta? | No - spasms will break their bones.
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| Scoliosis and PVR: | Elevated Pulmonary vascular resistance from chronic hypoxia causes Pulmonary HTN and Right ventricular hypertrophy.
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| What does scoliosis do to lung numbers? | Reduced lung volumes and chest wall compliance. PaO2 is reduced as a result of V/Q mismatch and increased PaCO2 signals severe disease.
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| Down Syndrome is an extra what? | Chromosome 21.
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| Down and catecholamines? | Decreased stores.
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| Obesity is an independent risk factor for: | DVT.
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| Obesity is associated with pseudotumor cerebri, which is what? | Benign intracranial hypertension.
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| What is maturity onset diabetes of youth? | Inherited form of NIDDM.
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| What's the most common childhood abdominal malignancy? | Wilm's tumor.
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| What is WAGR syndrome? | Airidia, genital anomalies, mental retardation, 30% chance of developing Wilm's tumor.
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| What causes prune belly syndrome? | It is caused by agenesis of the abdominal musculature, which results in a thin walled, protrudent abdomen.
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| Anesthetic implications for prune belly? | -Mult pulm complications with inability to cough effectively
-High risk of asp
-Controlled ventilation is necessary to prevent hypoventilation & hypoxemia
-Skeletal muscle relaxants are not needed
-Avoid overhydration
-Postop pulm complications
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| Phenylketonuria (PKU) is: | Disorder of amino acid metabolism.
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| PKU patients have: | -Most significant consequence is mental retardation
-At least ¼ of the patients have seizures
-Eczema is common
-Increased incidence of pyloric stenosis
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| PKU patients and drugs: | Very sensitive to narcotics and CNS depressants
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| Sickle cell results from: | Hemoglobin S results from the substitution of a valine molecule for a glutamate in position 6 of the beta chain.
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| What happens with hemaglobin S cells? | Altered hemoglobin readily forms polymers when deoxygenated producing the pathognomonic sickled RBC’s.
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| Genetic cause of sickle cell: | SCD – autosomal recessive sickling gene.
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| Full sickle cell patients have 90% HgbS and 10%: | HgbF.
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| People with sickle cell trait have up to how much HgbS? | 45%.
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| Infants under how many months will have false positive for sickle cell test? | 6 months.
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| What organ disorders in hemophilia? | Splenomegaly
Abnormal liver function
Joint disease related to hemarthrosis
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| Adequate clotting usually occurs with what percentage of clotting factors? | 30%
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| In patients with hemophilia and moderate bleeding, we should raise their clotting factors to what? | 40%
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| What do you give to Von Willebrand patients? | Factor 8 or DDAVP.
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| Intracellular C is primarily controlled by: | The sarcoplasmic reticulum and more specifically the ryanodine receptor.
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| MH is triggered by: | ALL the agents (except N2O) and sux.
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| MH specific signs: | Muscle Rigidity
Increased CO2 Production
Rhabdomyolysis
Marked Temperature Elevation
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| MH nonspecific signs: | Tachycardia
Tachypnea
Acidosis (Resp/Metabolic)
Hyperkalemia
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| What drugs for MH crisis? | Treat hyperkalemia (bicarbonate 1-2 mg/kg PRN)
Give dantrolene 2.5mg/kg push. Repeat PRN
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| CHD: Obstructive lesions: | Obstruction to blood flow is present. Obstruction may be intracardiac e.g. pulmonary or aortic stenosis or may be extracardiac e.g. Coarctation of the aorta.
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| What happens with increased pulm blood flow? | L to R shunting occurs (no cyanosis).
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| Typical L to R shunting patient and conditions? | These patients usually present with failure to thrive, pulmonary hypertension, recurrent chest infections or congestive cardiac failure(CCF). Examples include ASD, VSD or PDA.
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| Decreased pulm blood flow: | Right to left shunting occurs with some type of obstruction to normal pulmonary blood flow.
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| Presentation/examples of R to L shunting: | This results in cyanosis, polycythemia, and systemic emboli. Examples include tetralogy of Fallot, tricuspid atresia, and pulmonary atresia.
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| Shunting and inhalational induction: | R to L = DELAY uptake into blood occurs more slowly
L to R = minimal to no effect
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| Shunting and IV induction: | R to L = response is faster
L to R = minimal effect if systemic perfusion is preserved
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| 4 things in tet. of fallot: | VSD
Pulm stenosis
Overriding aorta
RVH
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