Spring 2011Mid 2 Chronic Peds For Pas

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primary care provider’s role as part of the team of providers caring for the child

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coordinating the further assessment of the child by the team of professionals that is indicated and provide continuity in the care of the child and family

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primary care provider’s counseling role

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Educating parents about normal and aberrant development and behavior may prevent problems through early detection and anticipatory guidance.... Many parents expect and need specific, detailed advice from the clinician

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Educational intervention begins in the home with early stimulation often with the aid of which specialists?

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childhood educator, nurse, or occupational/speech/physical therapist

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usually part of the overall educational program and is based on the tested language strengths and weaknesses of the child - should also have

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speech therapy, hearing testing

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nursing or social work involvement with the family. These specialties often have to coordinate other specialty services.

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Social and environmental intervention

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Medical intervention involves providing primary care and specific treatment of conditions associated with disability. Goal:

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minimize functional impairments in the absence of curative treatment (which is often not possible)

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vision impairment (we need to make the Dx around age

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4-8 mos. based on parental concerns

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PCPs need to screen children in infancy for hearing loss which is easy to do using

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Auditory Brainstem Response and Otoacoustic Emissions

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speech-language impairment: the PCP role is screening based on what test?

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Denver II

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provide for physical needs, emotional support, educations and socialization.

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family

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Regardless of family structure, the presence of a loving adult or adults serving as a parent or parents committed to the fulfilling of a child's physical, emotional, and socialization needs is the best predictor of

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a child's optimal health and development.

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Most common chronic dz of childhood. 1 in 13 school age children. 5 million children younger than 18. More than 5500 fatalities annually.

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asthma

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air pollution, temperature extremes, high humidity, allergens (like animal dander, molds, pollen), tobacco smoke, URI, exercise, rhinitis/sinusitis, GERD.

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Environmental triggers/factors for asthma

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PEFR of 80% - 100% of the child's personal best value

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Green zone: child is likely asymptomatic and should continue with meds as usual.

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PEFR 50% - 80% of the child's personal best value; coincides with more asthma symptoms;

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Yellow zone: rescue meds (ie. albuterol) are added; contact primary care provider if PEFRs do not return to green zone within the next 24 to 48 hours OR if asthma symptoms are deteriorating.

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PEFR of < 50% of child's best value

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MEDICAL EMERGENCY; rescue meds should be taken immediately; if PEFR remains in the red zone or significant airway compromise emergency care is needed and primary care provider called.

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most effective anti-inflammatory med for the tx of chronic, persistent asthma; available as metered-dose inhalers (MDIs), dry powder inhalers (DPIs), and nebulizer soln; egs. beclomethasone, budesonide, flunisolide, fluticasone, and triamcinolone.

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Inhaled Corticosteroids, long

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Oral, daily-use asthma meds that are potent mediators of inflammation and smooth muscle bronchoconstriction; zafirlukast is approved for children over 5 years. montelukast comes in granules or chewable tabs for children age 12 mos to 5 years.

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Leukotriene Modifiers, long

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formoterol
salmeterol;
often combined with inhaled corticosteroid
relax smooth muscle; dosed BID; egs.

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Long-Acting Beta2-Agonists, long

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not used as much anymore; considered as an alternative add-on tx to inhaled corticosteroids. mild to mod effictive as a bronchodilator; theophyline levels in blood have to be monitored closely and there are many adverse effects associated with this drug.

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Theophylline, long

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subcutaneous therapy with omalzumab (Xolair) is approved for mod to severe allergic asthma in children 12 yrs and older; it is a humanized anti-IgE monoclonal antibody.

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Novel Therapies

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albuterol, levalbuterol, and pirbuterol; most effective bronchodilators; work within 5 to 10 minutes and last 4 to 6 hours; usually prescribed for acute symptoms and as prophylaxis before allergen exposure and exercise; MDIs and nebulizers.

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Short-Acting Beta2-Agonists, quick

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ipratropium bromide
(Atrovent)
relieves bronchoconstriction
decreases mucus hypersecretion
counteracts cough-receptor irritability.

binds acetylcholine
at the
muscarinic receptors in
bronchial smooth muscle;
additive effect with
beta2-agonists

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Anticholinergic Agent, quick

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short bursts (3 to 10 days) are administered to children with acute asthma exacerbations; liquid or tablets; children with severe asthma may require oral corticosteroids for extended periods

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Oral Corticosteroids, quick

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Step-wise tx:

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Short-acting bronchodilator for all children with asthma, use rule of twos to determine if controller meds needed. reevaluate to step up or step down

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used to determine if a child has persistent asthma and needs daily anti-inflammatory meds; Daytime symptoms occurring > 2 times/wk or nighttime awakening > 2 times/month.

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Rule of Twos

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Susceptibility to DM I is controlled by

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alleles of Major Histocompatability Complex (MHC) II that express human leukocyte antigen (HLA).

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Genetic predisposition for DM type I is also associated with

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celiac disease and thyroiditis

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inappropriate polyuria in any child with poor weight gain, dehydration, or "the flu". Hyperglycemia, glycosuria, ketonuria, nonfasting glucose > 200

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mmon presentation of undiagnosed DM1

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% of children with undiagnosed DM I will present with DKA

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20-40

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obesity, acanthosis nigricans

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Type II Diabetes Mellitus (DM2) in children

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impermeability to Cl & excessive reabsorption of Na. dehydration, impaired mucociliary transport and airway obstruction. Most are colonized with H. influenza, S aureus, or P aeruginosa. exocrine pancreatic insufficiency

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cystic fibrosis (CF)

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symptom in CF that results from maldigestion

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steatorrhea

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Chronic bronchial infection and sinusitis. Digital clubbing, steatorrhea, meconium ileus, distal intestinal obstruction syndrome, insulin deficiency, allergic bronchopulmonary aspergillosis

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complications of CF

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lab test that is used to confirm the diagnosis of CF, and diagnostic level

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sweat test, >60 mEq

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these S/S may interfere with what test?Adult age, adrenal insuff., eczema, ectodermal dysplasia, nephrogenic DI, hypothyroidism, fucosidosis, mucopolysaccharides, dehydration, malnutrition, poor technique,

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possible causes for a false negative on cloride sweat test for CF

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Clearance techniques, Pharmacologic measures & ABX therapy.

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treatment for chest dz in CF.

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replace pancreatic enzymes, encourage high calories, vitamins, enzymes to increase absorption. too much lipase may result in intestinal obstruction though.

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treatment for pancreatic insufficiency in CF

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in newborn may require surgery, may use contrast enemas. In child, use balanced intestinal lavage solution via nasogastric tube or enema. Increase hydration, fiber

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treatment for Meconium ileus in CF

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tx w/ ursodiol or endoscopic dilation of the ampulla of Vater if stenosis is present.

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treatment for Cholestasis in CF

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sex-linked recessive trait, 2-3/ 10,000 boys. Absence of dystrophin, a protein that belongs in the muscle fiber plasma membrane

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Duchenne muscular dystrophy

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autosomal dominant genetic dz caused by progressive expansion of a triplet repeat in a gene designated myotonin protein kinase

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myotonic dystrophy

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2-3 yo develop awkward gait/run. Hypertrophy of the calf, mild-mod prox leg weakness. Child uses arms to climb up his legs and body (Gower sign). Arm weakness by 6 yo, w/c bound by 12yo. 16yo respiratory and arm weakness.

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Duchenne muscular dystrophy

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trouble relaxing mm contraction. hypotonia & weakness at birth or slowly progressive facial and distal ext weakness & myotonia at adolescence. arrhythmias, cataracts, male baldness, infertility. ptosis and drooping of lower lip.

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myotonic dystrophy

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treatment for myotonic dystrophy as well as Duchenne muscular dystrophy

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symptomatic

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Serum Creatine Phosphokinase levels are always elevated. Muscle Biopsy shows mm fiber degeneration & regeneration accompanied by increased intrafascicular connective tissue. dx by DNA probe.

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Duchenne muscular dystrophy

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Mat thyroid disorder,
FH mental retardation,
Low SES ,
Maternal seizures,
Eclampsia,
3rd trimester bleeding,
Multiparity,
Fetal growth retardation,
Abnormal presentation,
Congenital malformation,
hypoxic-ischemic or
bilirubin (kernicterus)
encephalopathy

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risk factors for CP
Cerebral Palsey

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epidemiology of CP

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2-2.5 of every 1000 live born children in developed countries

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Failure to reach motor milestones, asymmetric gross motor function, increased motor tone floppiness

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CP

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Results from injury of UMN of pyramidal tract. Occasionally bilateral. 2 of following:
abnormal movement pattern,
increased tone,
pathologic reflexes
(positive Babinski,
Hyperreflexia)

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Spastic CP

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Abnormal patterns of movement and involuntary, uncontrolled, recurring movements

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dyskinetic CP

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More than one type of motor pattern is present/one pattern does not clearly dominate another. sensory deficits, seizures, cognitive-perceptual impairments

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mixed CP

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management of the following diseases related to ??? may help improve educational attainment: Epilepsy, Learning difficulties, behavioral challenges, sensory impairments

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CP

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unknown etiology. higher risk in siblings compared to general population. males 4x more compared to females

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Autism

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normal growth rate until 1-2yo. not cuddly, with delayed or absent smiling. Solitary play w/ little to no interest in communication or eye contact. Intense, absorbing interests, ritualistic behavior, and compulsive routines necessary to avoid tantrums.

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autism

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CNS abnormalities, fragile-x syndrome, tuberous sclerosis, language disorders, Asperger dis., childhood disintegrative dis., Rett syndrome, pervasive developmental disorder not otherwise specified.

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ddx for autism

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tx is focused on sxs or syndromes. aggression may need atypical antipsychotics, inattention may need ADHD stimulant, self-stimulatory behavior may need naltrexone. Specialist referral necessary

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autism tx

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diverse, tends to run in some families- but no gene found, seen in children with other brain development disorders (ie fetal alcohol synd., Down synd.). common pathway for diverse causes, including genetic, organic, and environmental etiologies.

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Attention-Deficit/Hyperactivity Disorder (ADHD)

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Epidemiology of ADHD is what % of school age children? boys how many times higher than girls? 60-80% persist into adolescence, & some sxs into adulthood.

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8-10%, two times more likely

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in general, the sx are inattention, hyperactivity/impulsivity. What disorder? What quesitionairres are necessary?

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Conners Questionnaires and the Vanderbilt.

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why do a physical exam when suspecting ADHD?

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rule out underlying medical problems

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Behavioral Approaches- structure, routine, behavior goals. Stimulant Medication- tricyclic antidepressants, bupropion, methylphenidate, dextroamphetamine & clonidine. This is a tx for what disorder?

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ADHD