Acute Respiratory Distress Syndrome (ARDS): Pathophysiology and Clinical Manifestations, Exams of Pediatrics

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AC PNP Pediatric Nurse Practitioner - Acute

Care 2024 Board Exam Review Questions

and Answers 100% Pass

Certification - Answer>> Validation with objective testing Assent - Answer>> Consent obtained by a child 7 years or older for research Licensure - Answer>> Permission to practice granted by a specific organization Credentialing - Answer>> Verification of qualifications. Regulated by institution, mandated by joint commission. Having certification and licensure documented. Privileging - Answer>> Skills verification Scope of practice - Answer>> Determined by national organizations and guidelines, and controlled by states. Highest level of authority to determine your scope of practice is the institution where you work. Transition to extrauterine life- resp - Answer>> 1) reabsorb fetal lung fluid

2. sufficient lung surfactant leads to -increased FRC (lungs can expand) -decreased inspiratory pressure -alveolar filling -decreased PVR -increased blood flow to lungs Transition to extrauterine life- circulatory - Answer>> Decreased PVR leads to

-decreased pressure on R side -foramen ovale (hole between atria closes). Increased blood oxygen level and decreased PVR leads to ductus arteriosus closure. APGAR score - Answer>> *Measures HR, respiratory effort, muscle tone, reflexes, and color each with a 3 point scale from 0 to 2 with a high score of 10. Performed at 1 and 5 minutes of life. LGA infant - Answer>> ->90th percentile -can result in hypoglycemia, brachial plexus injury, fractured clavicles, skull injuries, facical palsy, polycythemia. SGA infant - Answer>> -<10th percentile -Can result in resp distress, perinatal asphyxia, meconium aspiration, poor feeding, bradycardia, hypoBG, hypothermia, polycythemia Preterm classifications - Answer>> -term: 37-40 weeks -preterm: <37 weeks gestation -late preterm: 34-36^6 weeks gestation -very preterm: <32 weeks -low birth weight: <2.5kg -VLBW: <1.5kg -ELBW: <1kg Health risks of preterm and LBW infants - Answer>> -resp distress, apnea of prematurity -increased susceptibility to infections -thermoregulation issues -PDA -NEC, GER -retinopathy of prematurity ROP -intraventricular hemorrhage IVH

-anemia -hernia/hydrocele/undiscended testes -hyperbili -decreased ability to concentrate urine leading to dehydration, -hypocalcemia -hypoglycemia Newborn nutritional needs (kcal) - Answer>> 100-110kcal/kg/day Newborn weight gain/loss - Answer>> -Newborns lose up to 10% of birth weight in first days of life. -Typical weight gain 0.5-1oz/day Newborn reflexes present at birth - Answer>> -sucking -rooting -moro (startle when dropped) -asymmetric tonic neck (arm outstretched on opposite side head is turned) -grasp Prenatal screening - Answer>> *First trimester screen b/w 11- 15 weeks combo of blood sample and US for genetic abnormalities. Blood testing offers genetic info about potential abnormalities, gender, other defects. Blood work is primary way to find out abnormalities. *US: size, singleton/multiple gestation, gross defects (polycystic kidney, cardiac, myelomeningocele) *Alpha fetoprotein -elevated levels may indicate neural tube defects, gastroschisis, omphalocele, chromosome abnormalities.

Maternal infections to ask about in history - Answer>> *Group B strep *STI *Rubella *Varicella Newborn screening - Answer>> *Varies by state *ideally performed around >24-48 hours of age then at 7-14 days *Hearing screen (at birth to within 1 mo of age) *Newborn cardiac screen -pulse oximetry -car seat study Infant development 1-3 months of age - Answer>> *1-3 mos of age -weight gain 0.5-1 oz/day -8 to 10 feedings/day -BMs: *Formula-fed: 1-2/day *BF: 3-5/day -Sleep: 16 hours/day *2 months- social smile *rear facing car seat until age 2 AND weight of car seat Infant exam- highlights - Answer>> *Ht, wt, HC on growth chart *abdominal and periodic breathing are normal findings for infants *Hernia (repaired around 1 yr of age- should be soft and reduceable), hydrocele, circumcised Infants 2-6 months of age - Answer>> *Doubles birth weight by 6 months of age *Posterior fontanel closes 1-3 months of age

*Obligate NOSE breathers (breath through their nose and if nose becomes occluded they have a hard time breathing through their mouth) *Mongolian spots- normal variation *Cafe au lait spots: may be faint at birth and more noticeable in first 2 years of life => associated with neurofibromatsosi, tuberous sclerosis, Fanconi anemia *hemangioma- may increase in size until around 6 months. Strawberry spots. If they are on eyes or eyelids, that is a referral. Infants- 4 months of age - Answer>> *Avg weight is 14-16 lbs (6.3-7.2 kg) *Can grasp and put things in mouth *Good head control, holds head up in tummy time *Begins to rollover both ways *laughs out loud, squeals Infants- 6 months of age - Answer>> *Babbles, imitates sounds *starts to sits without support or forward on hands *rolls from back to belly *holds bottle *smiles at self in mirror *briefly searches for lost object Infants- 9 to 12 months of age - Answer>> *9 months -responds to name -stranger anxiety -pull to stand and cruise -wave "bye bye" *12 months -stands alone, can walk with support, crawls well -knows name, 2 to 3 words -places objects in container -primitive reflexes disappear (moro, rooting, babinski)

-security blanket Infant red flags of development - Answer>> *0-1 mo: poor suck/swallow, sweating/fatigue w feeds, poor weight gain *3 mo: no attempt to raise head, increased/decreased tone, no hand-mouth activity, no turn to voice, no track *6 mo: does not begin to sit w/o support, no eye contact, no babble *9 mo: no self-feeding, no reciprocal vocalization (you say it they say it back) *12 mo: has not tripled birth weight, no pull to stand, no vocalization, no transfer of objects b/w hands, no point/gesture, walks on heels. Toddler: 15 mos to 2 yrs of age - Answer>> *15-18 mo: -Ant fontanel closes -responds to whisper test -shows affection, follows simple directions -physiologic control of sphincters is developing -scribbling -building 4 blocks -run, jump, climb stairs -drink from cup, spoon -temper tantrums -know 2-3 body parts -vocab of ~10 words; beginning 2 word sentences *safety: -falls common, choking common, head injuries common Toddler red flags - Answer>> *Lack of imitating sounds, eye contact, moving/exploring, disappearance of previously learned skills, crying for communication of wants. Preschool age 3-4 yrs - Answer>> *3 yrs -jump in place, ride tricycle, kick ball, build 8-10 block tower

-vocab 200+ words -knows own gender and learning others -sharing -friends -follows 2 to 3 step directions *4 yrs -hops/jumps on 1 foot -copies circle, builds tower of 10 blocks -concrete and egocentric -make believe, telling stories -cooperates with other kids -catches ball, does puzzle -lack concept of forever, see death as temporary. Interpret words literally and need less details. -Prepare for procedures a few hours ahead of time -Parental presence may reduce anxiety Preschool age 5 - Answer>> *speaks clearly *has friends, knows names *counts to 10+ *Can draw a person with 6+ parts, write name, recognize letters *copies shapes *begins to understand rules *red flags -unable to calm self -hair/eyelash pulling -stool holding -no pretend play -unintelligible speech -unable to count to 10 -easily distracted -cannot complete simple care activities (brush teeth)

School age 6-12 yrs - Answer>> *6-9yrs -increased motor strength, sports, bike riding -sharing, friends, right and wrong, follows rules, chores -understand concepts of space, cause/effect -read/write *12 yrs -writing and fine motor skills improve -best friends -know how people feel *Developing logic and reasoning skills *Understanding death as permanent *Rules and rituals are important *can participate in decision making Adolescent/Teen - Answer>> -show more concern about themselves -developing identity, social skills -emotions -less affection for caregivers -complex thought -relationships School age/teen red flags - Answer>> *Problems w/peer group- inability to make or retain friends -cruelty to animals/self -not using language to express feelings -acute/progressive decline in school performance -lack of interest in usual activities -severe behaviors Commonly tested conditions included in newborn screening test - Answer>> -Phenylketonuria (PKU): autosomal recessive, inability to break down amino acid phenylalanine

-Galactosemia (GAL): rare AR disorder, deficiency in enzyme needed to metabolize galactose in milk -Hypothyroidism -Hemoglobinopathies: hereditary, sickle cell anemia, thalassemia -congenital adrenal hyperplasia: AR, inborn deficiency of various enzymes necessary for biosynthesis of cortisol -maple syrup urine disease: inherited, inability to metabolize AAs -Homocystinuria: defiency of enzymes resulting in mental retardation. Diet treatment -Biotinidase deficiency -Toxoplasmosis: infection with parasite from maternal infection during pregnancy. -CF: AR, altered transport of chloride ions Dysmorphology in neonates - Answer>> *Most will have minor, however an infant with 3+minor can indicate an underlying major malformation or syndrome *Minor abnormalities are usually dysmorphic features commonly located on face, ears, hair, hands, and feet with no serious medical problems Infant MSK concern- unequal buttock skin creases - Answer>> Order hip US Lead screening - Answer>> *Lead level by 1 year of age; hemoglobin and/or hematocrit

When to transition to cup? - Answer>> 12 months of age Infant safety hazards - Answer>> *falls: stairs, windows *home proofing, firearms *burns/hot liquids *infant walkers unsafe *ingestions/poisonings/choking/suffocation When can potty training begin? - Answer>> 2-2.5 yrs of age When is control over elimination typically acheived? - Answer>> *daytime control: avg 28 months *nighttime control: avg 33 months Clinical assessment of nutritiion - Answer>> *Anthropometrics -weight and length (<2 yrs)/ height (<2 yrs) -HC (up to 3 yrs old) -skin fold measures -growth curves Ideal body weight equation - Answer>> *Actual weight/IBW x 100= %IBW Metabolic cart - Answer>> *Performed by an RT *Fio2 delivered must be <0.6; cannot be leaks around ETT or trach -Indirect measure of energy expediture to estimate calories needed during acute phase of illness to keep up with metabolic demand of illness. GIR equation - Answer>> (g/kg/day of glucose x1,000) divided by / 1,440 (min per day).

Laryngotracheobronchitis (Croup) - Answer>> *Inflammation/infection of subglottic airway, larynx, trachea, and bronchi; mucosal airway edema- decreased airway diameter and increased airway resistance. *signs and symptoms: Pre existing cold, barky cough, stridor, tachypnea, retractions *Etiology: -Paraflu type 1 and 2 most common -RSV, adenovirus, Influenza A and B, paraflu type 3 -mycoplasma pneumoniae (not common) *incidence: -children 3 mos to 5 yrs of age -most common in fall months -M>F Croup Dx - Answer>> *Symptoms, presentation and history, lateral neck film shows STEEPLE sign *Management: Dexamethasone 0.6 mg/kg IV or IM

• Do not give IM if child is stable (can be very painful) -oxygen and respiratory maneuvers if needed -other: Aerosolized racemic epinephrine, heliox (improves laminar flow b/c lighter gas), IV fluids if dehydrated -Otolaryngology consult if no improvement with standard therapy Tracheitis - Answer>> *most often bacterial *Stridor, tripod position, drooling, high fever >103 -H-flu, strep pneumioniae -lateral neck xray: THUMB sign -treatment: airway protection, broad spectrum abx, symptom management, steroids

-Difference b/w tracheitis and epiglotitis is where inflammation occurs (epitlottitis vs tracheitis). Usually more severe Tracheomalacia - Answer>> *Congenital anomaly of trachea, common in preemies. Can also be caused by previous injury, intubation, lesion. -Associated with feeding problems *Definition: upper airway (trachea) lacking cartilgaginous rigitidy (floppy). *clinical presentation: early in life, but symptoms may be delayed 2-3 months -expiratory stridor and cough: worsens with agitation, crying, feeding, upper airway infections -harsh barky cough. *Dx: by ENT with flexible bronchoscopy scope, CT scan, fluoroscopy, laryngoscopy *Management: observation (most cases), symptoms resolve at or <18 months -trach/cpap in worst cases -aortopexy for severe cases, suspends the anterior trachea reducing airway compression. Epiglottitis - Answer>> *Acute severe inflammation of epiglottitis, resulting in displacement posteriorly -may obstruct breathing -AIRWAY emergency. *Etiology: Haemophilus influenzae (most common), other bacteria/viruses (staph auerus, strep pneumoniae, Group A strep) *Epidemiology: most common 1-5 yrs of age

*Clinical manifestations: -sudden onset (sore throat, stridor, high fever, resp distress) -difficulty swallowing/drooling/dysphagia -muffled voice/hot potato voice -tripod positioning -anxious appearing child Epiglottitis dx and management - Answer>> *Dx: -lateral neck radiograph: enlarged epiglottis, distended hypopharnyx (thumbprint sign) -direct laryngoscopy: beefy red, swollen epiglottis -blood/throat cultures -CBC may reveal leukocytosis (non-specific) *Management -avoid noxious stimuli -consult ENT for intubation (HAVE OR READY) -Humidified oxygen until decision for reintubation -Abx: 7 to 10 days (3rd gen cephalosporin (ie ceftriaxone) or 3rd gen cephalosporin plus vancomycin if pencillin-resistant pneumococci or MRSA suspected) -consider systemic steroids -IV fluids to prevent dehydration. Retropharyngeal abscess - Answer>> *Definition: abscess in retropharyngeal space with trauma or infection *Children 1-5 yrs of age -pathogens: streptococcus pyogenes, staph aureus, and Haemophilus species *Presentation, history, findings: -recent intubation, oral foreign object, dental procedure, infection of structure draining into retropharyngeal space

-sore throat, fever, dysphagia, trismus, neck swelling -muffled voice -cervical adenopathy, retropharyngeal bugle, stridor, torticollis, drooling. Retropharyngeal abscess dx and management - Answer>> *Dx: -CBC with diff (leukocytosis), blood culture -lateral neck film: widening of retropharyngeal soft tissues and/or preverterbral space (may help differentiate RPA from epiglottitis). -CT neck with IV contrast: shows extent of infection, and differentiates from cellulitis. -chest xr: eval for medistinal expansion *Management; -avoid noxious stimuli, secure airway/intubate if necessary -surgical drainage: abscess >2cm, failure to improve on IV abx within 24 hours -broad spectrum abx, cover gram +/-, treatment variable, can change to oral Peritonsillar abscess - Answer>> *Definition: local cellulitis progresses first to phlegmon, then abscess; suppurative adenitis is most common. *Deep neck infection, more common in older kids/teens -common pathogens: streptococcus pyogenes, staphylococcus aureus, and haemophilus species (can be polymicrobial) *Clinical manfiestations: -sore throat, pain radiates to ear, tender cervical glands -fever, chills, facial swelling, drooling, halitosis -trismus; may refuse to eat or drink -voice may be muffled or difficult to understand swollen tonsils with uvula deviation (swollen tissues may obstruct airway, emergency!)

*Management: -broad spectrum abx for 7-10 days -refer to ED or ENT if not improving or not able to take PO. Obstructive sleep apnea - Answer>> *Hypertrophy of tonsils +/- adenoids Craniofacial and neurologic abnormalities= higher risk -micrognathia -maxillary hypoplasia -high arched palate -other midface defects Genetic associations: Trisomy 21, achondroplasia, Prader-Willi syndrome Clinical manifestations: -snoring (pauses/snorts) -difficulty breathing during sleep, difficulty staying awake during daytime -failure to thrive, obesity -severe: pulmonary hypertension, cor pulmonale OSA Dx/treatment - Answer>> Dx: clinical symptoms enlarged tonsils on exam -sleep study (gold standard) -consider ECHO and 12 lead ecg to r/o RVH *treatment: tonsillectomy and adenoidectomy (may not cure obese children) -causes of upper airway obstruction should be identified and managed -CPAP or BiPAP -Tracheotomy (curative, but reserved formost severe patients) Lower Airway Problems - Answer>> *Asthma

*Status asthmaticus *Pertussis *Pneumonia *Parapneumonic effusion/pleural effusion *Pneumothorax *Respiratory failure/ARDS *Cystic fibrosis *pulmonary embolism Asthma - Answer>> *Inflammation, bronchoconstriction, airway hyperresponsiveness *cough, wheeze, dyspnea, chest tightness, chest pain *Status asthmaticus: progressively worsening bronchospasm/airflow obstruction unresponsive to standard therapy Most common chronic illness in childhood *Triggers: -extrinsic: allergic/immunologic factors, exposures -intrinsic: infections -Exercise induced Asthma severity classification - Answer>> *Intermittent, mild persistent, mod persistent, severe persistent *Severity of chronic illness may assist in management of acute exacerbation *Sx/findings: cough, SOB, tachypnea, tachycardia, wheezing, prolonged expiratory phase, accessory muscle use, hypoxia?, pulsus paradoxus Status asthmaticus - Answer>> *DX:

-Chest radiograph: hyperinflation, flattened diaphragms, peribronchial thickening, narrowed cardiac silhouette -pulse ox, end tidal CO2, ABG, lactate, electrolytes *warning signs: -can't speak in full sentences -persistent hypoxia -normal or rising CO -continued demise despite treatment Status asthmaticus- initial management - Answer>> *Inhaled bronchodilator (MDI or nebulized) *Corticosteroids: 2mg/kg *O2 for hypoxia *cardiac and respiratory monitoring *pulse ox *Ask hx of admission/ICU care/intubated Status asthmaticus-additional care - Answer>> *Mg sulfate: calcium antagonist (smooth muscle relaxation). IV bolus. (Most common adverse reaction is hypotension) *CPAP, biPAP for hypoxia, intubation for respiratory failure (avoid at all costs- can trigger/worsen bronchospasm) *IV beta agonist (Terbutaline) infusion (need ECG monitoring, can cause arrythmias.) Methylxanthines (aminophylline, theophylline): narrow therapeutic index; requires serum drug level monitoring. High side effect profile. Admission criteria: persisting symptoms, O2 requirement -short acting beta agonists, either continuous or every 2-3 hours -prior intensive care admissions

Pertussis - Answer>> *Whooping cough *Etiology: -bordatella pertussis; aerobic gram negative coccobacillus -primarily toxin mediated disease: paralyzes cilia, causes inflammation -transmitted by aerosolized droplets, highly contagious -older children and adults are often the reservoir! *Stages:

1. catarrhal (first 1-2 weeks cough, rhinorrhea)
2. paroxysmal (whoop)
3. convalescent (persistent cough can last up to 10 weeks) Dx: -clinical history and symptoms -nasopharyngeal secretions (nucleic acid amplification tests NAATs, direct fluorescence antibody DFA, polymerase chain reaction PCR* -culture -CBC: **Leukocytosis (lymphocyte predominance 50-80k/cm -chest xr: unremarkable Pertussis management - Answer>> *Antibiotics -treat prior to dx confirmation -erythryomycin, clarithorymycin (>1 mo, must be PO), or azithromycin. Controversial: avoid erythromycin in infants < month of age for risk of hypertrophic pyloric stenosis -supportive care: hospitalization if PNA or risk for apnea, O2 and resp monitoring, humidification/suctioning, mechanical ventilation if significant apnea or alveolar disease *Complications: -pneumonia, atelectasis, OM, encephalopathy, seizures, death

-highest mortality rate- infants <6 months of age (only have 2 doses vaccine) Pneumonia - Answer>> Sx: significant history (*prolonged cold/URI (longer than 2 weeks), cough, wheezing) *Dx: -chest XR (not always needed, only if you can't get a good history/definitive PE) (Not recommended, determined by age/severity of illness) -Severe disease/toxic appearance-CBC, CRP, cold agglutins, electrolytes, blood culture, mycoplasma culture. -inflammatory markers do not differentiate viral vs bacterial etiology; can be used for trends in children with severe illness *If you did do a chest XR, findings: -bacterial: patchy infiltrates, pleural effusion, atelectasis -viral: perihilar streaking, increased interstitial markings, peribronchial cuffing or patches Pneumonia etiology - Answer>> *Determined by child's age and contributing factors -neonates: most often bacterial; associated with pathogens in birth canal (Group B strep, Klebsiella, e.coli, listeria monocytogenes (one of the reasons you use ampicillin to treat a neonate), chlamydia) -late onset neonatal: staphylococcus, streptococcus -older infants/toddlers (30d to 2y): viral -2 to 5 yrs: virus still common, increase in streptococcus pneumoniae and Haemophilus influenzae type B (HIb)

-5 to 13 yrs of age: myocplasma pneumonia, strep pneumoniae most common. Pneumonia treatment - Answer>> *Targeted for expected pathogen based on child's age and severity of illness. *Abx (if indicated) -neonates: ampicillin plus aminoglycosides or 3rd gen cephalosporin, EES or arithromycin for chlamydia) -infants >3 months and young kids: high dose amoxicillin or beta lactam, IV ampicillin, penicillin -school age: macrolide; pneumococcal: PCN, clindamycin -aspiration pneumonia: clindamycin (length of treatment no longer than 7 days) *respiratory supportive measures as indicated by exam *Antipyretics *fluid management as needed. Pneumonia requiring hospitalization - Answer>> *toxic appearance *predisposing underlying condition: expect more serious course *complications (ex: effusion/empyema); need for IV abx *Suspicion or confirmation that CAP is due to a pathogen with increased virulence (staph, group A strep) *Oxygen requirement *high flow or biPAP Parapneumonic effusion vs empyema - Answer>> *Parapneumonic effusion: bacterial pneumonia usually cause -small effusion will resolve with appropriate diuretics -if moderate/lg will need drainage

-commonly transudative. pleura inflamed, leakage of proteins. Fluid is sterile with low leukocyte count *Empyema (pus): -spread of infection (bacteria) into pleural fluid. -fluid is purulent when drained -Abx mainstay of therapy. May require thoractomy/chest tube +/- fibrinolytic admin, video assisted thoracentesis (VATs) (for loculated empyema) Lateral decubitus XR - Answer>> *Helpful with determining if fluid is present (Pleural effusion) *Fluid is dependent like water in a glass *US can show fluid layers and guide CT placement *CXR should always be done after CT to ensure that you are not causing a pneumothorax Pneumothorax - Answer>> *Air leak into pleural space *acute sx: SOB, tachypnea, chest pain -athletes, children with asthma (status asthmaticus) -can result from trauma to chest *Clinical findings: tachypnea, displaced (R side of chest) or muffled heart sounds, crepitus *Chest XR: air leak (air shows up dark outside of lung fields) *Treatment- depends on size and sx -100% O2 -needle chest, chest tube insertion Acute Respiratory Distress Syndrome - Answer>> *Result of injury to the alevolar capillary membrane *Etiology: preceded by a variety of disease processes -direct lung injury -shock (any kind) -sepsis

-drowning -trauma -aspiration -pulmonary contusion -transfusion related lung injury (TRALI) -cardiopulmonary arrest *Clinical manifestations: -tachypnea, tachycardia -respiratory distress, hypoxia (significant) -hypocapnia initially -decreased aeration -crackles, wheezing ARDS stages - Answer>> 1) Exudative -injury to alveolar capillary barrier -increased pulmonary congestion and pulmonary edema -depletion of endogenous surfactant

2. Proliferative -proliferation of type 2 pneumacytes, fibroblasts, and myofibroblasts -healing begins -heterogenous lungs- interspersed with normal lung function
3. Fibrotic -lungs begin to remodel with collagenous tissue -fibrotic scarring -complications of treatment begin to surface *progressing through these stages leads to pulmonary hypertension, bronchoconstriction, hypovolemia, and increased PVR

Cystic Fibrosis - Answer>> *Autosomal recessive genetic disease, identified in newborn screening, caused by mutations in the CF transmembrane regulator gene, many mutations. -defective/abnormal gene -impairs movement of salt and water across the epithelial cell wall in the exocrine glands- sticky secretions. -body systems: lungs, pancreas, intestine, liver, sinuses, reproductive tract, sweat glands *presentation: -Neonatal presentation includes meconium ileus, neonatal bowel obstruction, poor growth, prolonged jaundice -Post natal: frequent lower respiratory tract infections *DX: -prenatal and neonatal screening (does not test for all gene types) -post natal: sweat chloride testing (initial ***), confirmation testing with 2nd sweat testing or genotyping. Some centers perform nasal potential difference, or gene testing Cystic Fibrosis clinical manifestations - Answer>> *Progressive pulmonary disease *Pulmonary exacerbations -increased cough -sputum production -hemoptysis -decreased pulmonary function (PNA, infections) *Allergic bronchopulmonary aspergilliosis -nasal polyposis, sinusitis -poor growth development -GER -fat soluble vitamin deficiency (ADEK) -distal instestinal obstruction; chronic constipation -elevated liver function tests (gallstones, cirrhosis) -infertility (men: absence of vas deferens)

CF management - Answer>> *Resp: -airway clearance techniques, hydrators (dornase alpha, hypertonic saline) -abx for gram negative organisms with PNA or tracheitis *Nutritional therapy: -high cal diet, unrestricted fat, pancreatic enzymes -may need G tube feeds to supp *Endocrine: -frequent blood glucose monitoring, may require insulin *Hepatic: -improves with urosdeoxycholic acid (ursodiol) -some require liver tx *Prognosis: -90% deaths occur d/t resp failure -median life expectancy increasing (~37 y) Pulmonary Embolism - Answer>> *Occlusion in the pulmonary arterial bed (thromboembolism most common) *More common in obese teens on OCP, following dx of DVT Clinical manifestations: pleuritic chest pain, dyspnea, cough, crackles, wheezing, hemoptysis -tachycardia, fever, diaphoresis -sense of doom -phlebitis -hemodynamic instability in severe cases Dx: -respiratory alkalosis, arterial hypoxemia, or normal -ELEVATED D-DIMERS -imaging: CXR may be normal, ventilation perfusion scan (normal does not exclude possibility, reports are interpreted in terms of

probabilty), helical spiral CT scan (results somewhat dependent on radiologist experience), pulmonary angiography) PE management - Answer>> *ABCs Vasopressor support if indicated Anticoagulation (heparin, LMWH, lovenox, coumadin) *Thromboylsis (urokinase, sreptokinase, recombinant tissue plasmonigen activator) *Thrombectomy, if persistent hemodynamic compromise *Inferior vena cava filters *Consult hematology Transesophageal fistula (TEF) - Answer>> *Congenital or acquired communication between the trachea and esophagus *Several types based on location of fistula (five types) *Congenital malformation -acquired forms (blunt chest or neck trauma, excessive pressure in tube cuff pressure in intubated infants) *DX: -prenatal: US -Post: XR -Acquired: instillation of contrast media in to esophagus or during flexible endoscopy/bronchoscopy) *Mgmt: -presurgical: prevent aspiration -surgical intervention Causes of respiratory acidosis - Answer>> -hypoventilation -CNS depression (anesthesia, narcs, sedatives, drug OD) -respiratory neuromuscular disorders -trauma: spine, brain, chest wall -restrictive lung diseases -chronic obstructive pulmonary disease