Pedia2 Le6 Handout

PEDIATRICS2 – LE6 Handout [by: msredlips ] 1

FEVER WITHOUT A FOCUS (Dra. Morelos) FEVER

• Controlled increase in body temperature over normal values • °T >38°C rectal • Normal 36.6°C – 37.0°C rectal

HYPERTHERMIA

• °T >40°C o Central o Heat stroke o Malignant hyperthermia o Drug fever

REGULATION OF BODY TEMPERATURE

• Preoptic and Anterior Hypothalamus • Thermosensitive neurons • Normal Body Temperature • 36.6°C – 37.9°C rectal • Diurnal variation (Circadian Rhythm)

PATHOGENESIS OF FEVER

• 3 Mechanisms of Fever • The body’s thermostat is reset at a higher temperature

o In response to Pyrogens o Heat production > heat loss o Defective mechanisms of heat loss

PYROGENS

• Endogenous Pyrogens o Cytokines IL1 and IL2 o TNF α o Interferon β and γ o Lipid mediator PGE2

• Exogenous Pyrogens • Infectious pathogens

o Microbes and toxins – stimulate production of endogenous pyrogens

o Endotoxins • Other substances that stimulate endogenous pyrogens

o Antigen-‐antibody complexes o Complement o Lymphocyte products o Androgenic steroid metabolites o Vancomycin, Amphotericin B, Allopurinol

• Producers of endogenous pyrogens o Malignancies o Inflammatory Diseases

EFFECTS OF FEVER

• ↑O2 Consumption • ↑CO2 Production • ↑Cardiac Output • ↑Risk for seizures

ETIOLOGY OF FEVER

• Infections • Inflammatory disorders • Neoplasms • Miscellaneous • The most common cause of acute fever are self-‐limited viral

infections and uncomplicated bacterial infections

FEVER PATTERNS • Slow decline of fever over a week – viral • Prompt resolution of fever after effective antimicrobial tx –

bacterial • Intermittent fever -‐ normalizes • Septic/hectic fever – extremely wide fluctuations of fever • Sustained fever – does not vary by >0.5°C • Remittent fever – varies by >0.5°C (never normalize) • Relapsing fever – with intervals of normal temperature • Tertian fever – 1st and 3rd (P Vivax) • Quartan fever – 1st and 4th (P malariae) • Biphasic fever – camel back fever pattern in Dengue, polio,

leptospirosis • Periodic fever – regular periodicity • Factitious fever – self induced • Double quotidian fever – peaks twice in 24 hours, with

arthritic fevers • Single isolated fever spike – transfusion reaction, catheter

placement (foreign body reaction), colonization of infected body surface

EVALUATION OF ACUTE FEVER

• History • PE • Labs

TREATMENT OF ACUTE FEVER

• Tx of self-‐limiting illness • Tx for certain conditions only

o Fever <39°C in healthy o High risk groups o Tx of underlying etiology

• Antipyretics FEVER WITHOUT A FOCUS

• Rectal temperature of ≥38°C as the sole presentation o Fever without localizing signs o Fever of unknown origin

FEVER WITHOUT LOCALIZING SIGNS

• etiology and evaluation of fever without localizing signs depends on the age of the child

o neonates or infants -‐ 1 mo of age o infants >1 mo -‐ 3 mo of age o children >3 mo -‐ 3 yr of age

• Low risk vs High risk • Febrile patients at risk for serious bacterial infections

(immunocompetent) o Neonates <28days o Infants 1-‐3mos o Children 3-‐36 mos o Hyperpyrexia >40°C o Fever with petechiae

• Febrile patients at risk for serious bacterial infections (immunocompromised)

o Sickle cell disease o Asplenia o Complement/Properdin deficiency o Agammaglobulinemia o AIDS o Congenital heart disease (infective endocarditis is

most feared, also brain abscess with left to right shunting)

o Central venous line

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PEDIATRICS2 – LE6 Handout [by: msredlips ]

o Malignancy • “Toxic”

o Clinical picture consistent with sepsis syndrome: § lethargy § signs of poor perfusion § cyanosis § hypoventilation § hyperventilation

• all febrile children less than 36 months of age who are toxic-‐appearing should be hospitalized for evaluation and treatment of possible sepsis or meningitis

NEONATES: FEVER W/O LOCALIZING SIGNS

• All should be hospitalized • difficult to clinically distinguish between a serious bacterial

infection and self-‐limited viral illness • Blood, Urine, Stool, CSF cultures/GS • Empirical IV antiviral / combination antibiotics

1-‐3MOS: FEVER W/O LOCALIZING SIGNS

• Etiology o Viral o Bacterial o GBS o L. monocytogenes

o Salmonella o E. coli o N.meningitidis o S.pneumoniae o Hib o Staph aureus

• low risk o Normal physical examination o Uncomplicated medical history o Normal labs

§ Urine: ü <10 WBC/HPF, (-‐)nitrite ü (-‐)leukocyte esterase

o Stool studies if diarrhea (-‐) RBC and <5 WBC/HPF o CSF cell count <8 WBC/mm3 (-‐) Gram stain o Peripheral blood: 5,000-‐15,000 WBC/mm3 <1,500

bands or band:total neutrophil ratio <0.2 o Chest radiograph without infiltrate

• If child fulfills all low-‐risk criteria administer no antibiotics • Ensure follow-‐up in 24 hr and access to emergency care if

child deteriorates. • Daily follow-‐up should occur until blood, urine, and CSF

cultures are final. • If any cultures are positive, child returns for further

evaluation and treatment. • If child does not fulfill all low-‐risk criteria: hospitalize. • Administer parenteral antibiotics until all cultures are final

until definitive diagnosis is determined and until child is adequately treated.

3-‐36 MOS. FEVER WITHOUT LOCALIZING SIGNS

• Approximately 30% of febrile children have no localizing signs of infection

• Etiology o Viral o Bacterial (occult bacteremia)

§ S. pneumoniae § H. influenzae type B

§ N. meningitidis § Salmonella, S. aureus. S. pyogenes

3-‐36 mos. Fever 38°-‐39°C without localizing signs (low risk) Reassure: diagnosis is most likely a self-‐limiting viral infection

• no antibiotics • Acetaminophen 15mg/kg q 4hrs • advise return • with persistence of fever

o If temperatures >39°C • With appearance of new signs and symptoms • Risk factors for occult bacteremia

o temperature ≥39°C o WBC count ≥15,000/µL o ↑absolute neutrophil count o ↑band count o ↑C-‐reactive protein

3-‐36 mos. Fever ≥39°C without localizing signs Sequelae of occult bacteremia

• meningitis, cellulitis, pneumonia, pericarditis, epiglotitis,jt.infections, osteomyelitis, suppurative arthritis, otitis media, sinusitis, enteritis, urinary tract infection

SEPTIC WORKUP

• Urine culture for Males <6 mo of age Females <2 y of age • Stool culture if Blood and mucus in stool or 5 WBCs/hpf in

stool • Chest radiograph if with Dyspnea, tachypnea, rales, or

decreased breath sounds • Blood culture

o Option 1: All children with temperature ≥39°C o Option 2: Temperature ≥39°C and WBC count ≥15

000 • Lumbar puncture

o Clinician determines LP based on hx, observational assessment, and PE

• Blood culture o (+) Streptococcus pneumoniae,

§ Persistent fever: Admit for sepsis evaluation and parenteral antibiotics pending results.

§ All others: Admit for sepsis evaluation and parenteral antibiotics pending results.

• Urinalysis (+) All organisms: o Admit if febrile or ill-‐appearing

• Outpatient antibiotics if afebrile and well. • Empiric antibiotic therapy

o Option 1: All children with temperature ≥39°C o Option 2: Temperature ≥39°C and WBC count ≥15

000 • Acetaminophen 15 mg/kg per dose every 4 h for

temperature ≥39°C FUO: FEVER OF UNKNOWN ORIGIN

• fever documented by a health care provider and for which the cause could not be identified after 3 wks of evaluation as an outpatient or after 1 wk of evaluation in the hospital

§ FUO Etiology o Infections 36% o Neoplastic diseases 19% o Collagen Vascular Diseases/Autoimmune Disorders


13% o No Diagnosis 7% o FUO is more likely an unusual presentation of a

common disease than an uncommon disease. FUO: 4 SUBTYPES

• Classic FUO • Health-‐Care associated FUO • Immunedeficient FUO • HIV-‐related FUO

FUO: DIAGNOSTIC CONSIDERATIONS

• Abscesses • Bacterial disease • Localized infections • Fungal disease • Parasitic disease • Viral disease • Rheumatologic disease • Hypersensitivity disease • Neoplasms • Granulomatous disease • Familial/hereditary diseases • Drug Fever • Miscellaneous

DRUG FEVER

• Onset : 7-‐10days after start of tx • Hectic fever with rash/eosinophilia but with a preserved

sense of well being (20%) • DX: resolution of fever in 48 hrs after d/c of drug, or 3 to 5

drug T/2, whichever is longer; recrudescent fever occurs within a few hours after the drug is restarted

FUO : HISTORY

• Age • Exposure to wild or domestic animals – zoonotic diseases • Pica • Unusual dietary habits • Travel and prophylactic immunizations

FUO :PE

• General appearance, including sweating during fever • Ophthalmic examination • Sinuses • Pharynx • Thyroid • Cervical lymph nodes • Murmur • Abdomen • muscles and bones should be palpated • Rectal exam • Skin and nails

FUO: LABORATORY EVALUATION

• CBC • Peripheral blood smear • Urinalysis • ESR CRP ANA RF • Cultures • Tuberculin test • Radiographic examinations • Bone marrow examination • Serologic tests

• Radionuclide scans • Echocardiogram • Ultrasonography • CT scan/ MRI • Biopsy • Bronchoscopy, Laparoscopy, Endoscopy

FUO: TREATMENT • Therapy should be withheld until the cause has been

determined. • The ultimate treatment of FUO is tailored to the underlying

diagnosis. • Avoid empirical trials. • After a complete evaluation antipyretics may be indicated to

control fever and relieve symptoms. FUO: PROGNOSIS

• The prognosis of FUO is determined by the cause of the fever and by the nature of any underlying disease or diseases.

• Children with FUO have a better prognosis than do adults. • Patients in whom FUO remains undiagnosed after extensive

evaluation generally have a favorable outcome, characteristically with resolution of their fever in 4 or more weeks without sequelae

CHRONIC ILLNESSES (Dra. Morada) EPILEPSY

• It is a disorder of the brain • Characterized by:

o enduring predisposition to generate seizures o neurobiologic, cognitive, psychological, and social

consequences • Present when >2 unprovoked seizures occur in a time frame

of >24 hr. • The occurrence of 1 seizure or of a febrile seizure does not

necessarily imply dx of epilepsy Clinical dx

• At least 1 unprovoked epileptic seizure with either a second such sizure

Seizure disorder

• Any one of several disorders: o Epilepsy o Febrile seizure o Single seizure o Seizures due to metabolic, infectious, hypocalcemia

Evaluation of 1st seizure

• Adequacy of aiway, ventilation and cardiac function • Temp, BP, glucose • Infection, head trauma, drugs/toxins • History • Focal or generalized?

Description of seizure

• Tonic = increased rigidity • Atonic = flaccidity • Clonic = rhythmic muscle contraction and relaxation • Myoclonus = shocklike contraction of a muscle • Aura: epigastric pain

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Treatment of epilepsy • Ensure that a patient has a seizure disorder and not a

condition that mimics epilepsy (migraine, tuberous sclerosis, movement disorders, encephalopathies)

• If (-‐) EEG and neuro exam : watchful waiting • Recurrent seizure: start anticonvulsant • DOC: depends on classification of seizure

CEREBRAL PALSY

• Permanent disorders of movement and posture causing activity limitation

• Attributed to nonprogressive disturbances in the developing fetal or infant brain

• Static encephalopathy EPIDEMIOLOGY & ETIOLOGY

• Most common and costly form of chronic motor disability • 3.6/1000 incidence • 1.4/1 male/female ratio • Born term, uncomplicated • Genetic: interleukin 6 • Premature: ICH and periventricular leukomalacia (PVL) • Infertility treatments

CLINICAL MANIFESTATIONS

• Spastic hemiplegia – decreased spontaneous movements on the affected side

• Spastic diplegia – bilateral spasticity of the legs that is greater than in the arms

• Spastic quadriplegia – marked motor impairment of all extremities

• Athetoid – hypotonic with poor head control; marked rigidity DIAGNOSIS

• Hx and PE: progressive disorder of the CNS • MRI scan of the brain • Tests of hearing and visual function • Genetic evaluation

TREATMENT

• Recent study: prenatal treatment of mothers with magnesium

• Prevent development of contractures • Multidisciplinary • Treat spasticity: benzodiazepines

DIABETES MELLITUS

• Metabolic syndrome characterized by hyperglycemia • Type 1 DM = caused by deficiency of insulin secretion due to

pancreatic B-‐cell damage • Type 2 DM = caused by insulin resistance at skeletal muscle,

liver, adipose tissue


Long-‐term Complications • Retinopathy • Nephropathy • Neuropathy • Ischemic heart disease • Arterial obstruction with gangrene of the extremities

TYPE 1 DM (Immune Mediated)

• Boys = girls • Peak: 5-‐7 yr of age & puberty • + family history but 85% of newly diagnosed do not have

family history • Environmental factors: viral infections, mumps virus, etc

PATHOGENESIS

• Autoimmunity • A genetically susceptible host develops autoimmunity against

his own B cells. CLINICAL MANIFESTATIONS

• Hyperglycemia • Intermittent polyuria and nocturia • Polydipsia • Monilial vaginitis • Weight loss • Abdominal discomfort, nausea, emesis • Dehydration • Polyuria • Kussmaul respirations, fruity breath color, diminished

neurocognitive function, coma DIAGNOSIS

• Inappropriate polyuria in any child with dehydration, poor weight gain, or “the flu”

• Hyperglycemia • Glycosuria, ketonuria • Nonfasting blood glucose >200 mg/dL (with or without

ketonuria • Electrolyte abnormalities • Baseline HbA1c

DIABETIC KETOACIDOSIS

• Occurs in 20-‐40% with new onset DM • Mild: oriented, alert but fatigued • Moderate: Kussmaul respirations; oriented but sleepy;

arousable • Severe: Kussmaul or depressed respirations; sleepy to

depressed sensorium to coma TREATMENT

• Insulin therapy • Diabetes education – monitoring of blood glucose, etc • DKA protocol – see Table 583-‐4, 19th ed Nelson • Cerebral edema –the major cause of morbidity and mortality • Nutritional management • Monitoring

BRONCHIAL ASTHMA

• Airways hyperresponsiveness to provocative exposures • Inflammatory condition

ETIOLOGY • Genetics

o Interleukin-‐4 gene o ADAM 33

• Environment o Allergens, infections, microbes, pollutants, stress

EPIDEMIOLOGY

• Boys • Children in poor families • Ethnic minorities, urban living

TYPES OF CHILDHOOD ASTHMA

1. Recurrent wheezing 2. Chronic asthma 3. Females who experience obesity and early-‐onset puberty

PATHOGENESIS

• Airflow obstruction • Small airways: airflow is regulated by smooth muscle

encircling the lumens; bronchoconstriction of muscular bands • Cellular inflammatory infiltrate and exudates • Breach in normal immune regulation • Mucus hypersecretion

CLINICAL MANIFESTATIONS

• Intermittent dry coughing • Expiratory wheezing • Older: shortness of breath/chest tightness • Younger: chest pain • Lack of improvement with bronchodilator – inconsistent with

asthma Other findings

• Decreased breath sounds (R lower posterior lobe) • Crackles and rhonchi • Labored breathing • Insp and exp wheezing, poor air entry, suprasternal and

intercostal retractions, nasal flaring, accessory respiratory muscle use

ASTHMA TRIGGERS

• Viral infections of the respiratory tract • Aeroallergens: animal dander, indoor allergens, dust mites,

cockroaches, molds • Seasonal aeroallergens: pollens and molds • Tobacco smoke • Air pollutants: dust, etc • Strong or noxious odors or fumes • Occupational exposures: paint, etc • Cold air, dry air • Exercise • Crying, laughter, hyperventilation • Co-‐morbid conditions: rhinitis , sinusitis, GER

DIAGNOSIS

• History • PE • Improvement with bronchodilator • Asthma triggers • Environmental history

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DIFFERENTIAL DIAGNOSIS: Upper Respiratory Tract Conditions

• Allergic rhinitis • Chronic rhinitis • Sinusitis

Middle Respiratory Tract Conditions

• Laryngotracheobonchomalacia • Pertussis • Vocal cord dysfunction • Foreign body aspiration • Chronic bronchitis (tobacco smoke)

Lower Respiratory Tract Infections

• Viral bronchiolitis • Gastroesophageal reflux • Tuberculosis • Pneumonia • Pulmonary edema (e.g. congestive heart failure)

LABORATORY

• Pulmonary Function Testing o Spirometry o Bronchoprovocation challenges o Exercise challenges o Peak expiratory flow (PEF) monitoring

• Radiology TREATMENT 1: Regular Assessment and Monitoring

• Severity o Age group: 0-‐4 yr, 5-‐11 yr, >12 yr

1. Intermittent Asthma -‐ <2 days/wk sx 2. Persistent Asthma

a. Mild -‐ >2 days/wk but not daily b. Moderate -‐ daily c. Severe – throughout the day

• Control o Well-‐controlled: sx <2 days/wk but not more than

once on each day o Not well-‐controlled: sx >2 days/wk or multiple

times on <2 days/wk o Very poorly controlled: sx throughout the day

• Responsiveness to therapy o The ease with which asthma control is attained by

treatment o Can encompass monitoring for adverse effects

related to medication use 2: Patient Education

• Written asthma management plan o Daily “routine” management plan o Action plan for asthma exacerbations

• Regular follow-‐up visits: o 2x yearly (more often if asthma not well controlled o Monitor lung function annually o CAMP = Childhood Asthma Management Program

3: Control of Factors Contributing to Asthma Severity

• Eliminating and reducing problematic environmental exposures

• Treat co-‐morbid conditions

o Rhinitis o Sinusitis o GER

4: Pharmacotherapy

• Long-‐Term Controller Medications • Quick-‐Reliever Medications • “Step-‐Up, Step-‐Down” Approach

Long-‐Term Controller Medications

1. Inhaled corticosteroids (Fluticasone) 2. Systemic corticosteroids 3. Long-‐acting inhaled B-‐agonists (LABAs) (Salmeterol) 4. Leukotriene-‐modifying agents (Montelukast) 5. Nonsteroidal anti-‐inflammatory agents (Cromolyn) 6. Theophylline 7. Anti-‐immunoglobulin E (Omalizumab)

Quick-‐Reliever Medications

1. Short-‐acting inhaled B-‐agonists (SABA): Albuterol, Terbutaline

2. Anticholinergic agents: Ipratropium bromide 3. Short-‐course systemic corticosteroids (Prednisone)

Delivery Devices

• Metered-‐dose inhaler (MDI) o Dry powder inhaler (DPI): Diskus, Twisthaler o Suspension/solution via nebulizer

• Spacer devices o For preschool-‐aged children

Inhalation Technique

• For each puff of MDI-‐delivered medication = slow (5-‐sec) inhalation, then a 5-‐ to 10-‐sec breath-‐hold

• Spacer/mask: each puff for about 30 sec with regular breathing

PROGNOSIS

• 35% of preschool-‐aged children have recurrent wheezing • 1/3 persistent asthma • 2/3 improve • Asthma severity by 7-‐10 y/o is predictive of asthma

persistence in adults PREVENTION

• Cornerstone of asthma control = anti-‐inflammatory interventions

• Avoidance of tobacco smoke • Prolonged breastfeeding (>4 mo) • Active lifestyle • Healthy diet

CHRONIC ILLNESSES in CHILDHOOD (Dra. SG Aro) DEFINITION

• Chronic: of long duration • Special: distinctive, exceptional, • Disability: incapacity, handicap

• CHRONIC ILLNESS -‐ defined by its duration and nature; health

condition that persists longer than 3 months • FUNCTIONAL LIMITATION – specific impact on function


• DISABILITY – social impact of a condition on a child’s daily life; assessed by measuring limitations (participate in daily activities, school performance, attendance,community functions)

CHRONIC ILLNESS

• Complex and dynamic • Rare and heterogenous • In infancy, affects both growth and development • Failure to thrive – common manifestation affecting feeding

and metabolic demands • Boys have higher rates of chronic illness than do girls • Contributes to social disparities in child health

Differences Between Acute & Chronic Illness Acute Chronic Onset abrupt usually graduated Duration limited lengthy, indefinite Cause single multiple, changes Diagnosis usually accurate often uncertain Prognosis usually accurate often uncertain Intervention usually effective often indecisive Outcome cure no cure Uncertainty minimal pervasive The Impact of Chronic Illness – The Individual

• Initial Impact o Shock o Denial o Loss and grief o Anxiety and depression

§ 20-‐25% experience psychological symptoms • If these reactions last too long, they can have an negative

effect on the illness • Must adjust to:

o Symptoms of the disease o Stress of Treatment o Feelings of vulnerability o Loss of Control o Threat to self-‐esteem o Financial Concerns o Changes in family structure

Chronic Illness as a Crisis

• Illness is a crisis because it is a turning point in an individual’s life.

• Disruption to established patterns of personal and social functioning produces a state of psychological, social, and physical disequilibrium

• Adaptation = finding new ways of coping with drastically altered circumstances. Restore equilibrium.

Crisis Theory (Moos, 1982)

• A model describing the factors that affect people’s adjustment to having serious illness.

• Coping process (3 stages) is influenced by 3 factors o Illness-‐Related Factors o Background and personal Factors o Physical and Social Environment Factors

• Coping process influences outcome of crisis

Crisis Theory of Chronic Illness – A Model

Contributing Factors

• Illness-‐Related Factors o Degree of illness acceptance o Degree of lifestyle/functional impairment

• Background and Personal Factors o Demographic -‐ Age, Gender, SES o Personality -‐ Negative affectivity vs. Hardiness

• Physical and Social Environment Factors o Social support – Instrumental & Emotional

§ In the long run emotional is better The Coping Process

• Cognitive appraisal o Meaning or significance of the illness o Threat and coping ability

• Adaptive tasks o Formulation of tasks to help cope with illness

§ Illness-‐related – Impairment, treatment, hospital

§ General psychosocial functioning – Self-‐perception, self-‐esteem

• Coping skills o Denial, information seeking, goal setting, recruiting

support, catharsis Outcome of Crisis

• Adaptation and Adjustment o Physical, vocational, self-‐concept, social, emotional,

compliance • Quality of Life

o Degree of quality people appraise their lives to contain § Quality = fulfillment or purpose

o Health-‐related quality of life (physical status and functioning, psychological status, social functioning, disease or treatment-‐related symptomatology)

The Impact of Chronic Illness -‐ The Family

• Must adjust to: o Increased stress o Change in the nature of the relationship o Change in family structure/roles o Lost income all have impact

Parental Responses to Illness or Disability

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Family Centered Approach & Developmental Approach

• Infancy • Toddlerhood • Preschool • SchoolAge • Adolescence • Trust • Autonomy • Initiative • Industry/Accomplishment • Identity

Assessing Family Strengths

• Available Support Systems • Perception of the illness/disability • Coping Mechanisms • Available Resources • Concurrent Stresses

Tasks of Parents of Children with Chronic Conditions

1. Accepting the Child’s Condition “It’s not the hand you’re dealt, but how you play your cards”

2. Managing Day-‐to-‐Day • Constant Attention • Details/preplanning • Rxns of other children • Social Relationships • Effects on Siblings • Marital Relationships

3. Meeting the Child’s Normal Developmental Needs 4. Meeting Developmental needs of others in the Family

• Sibling issues • Parental roles • Single parenting • Normalcy • Extended family

5. Coping with ongoing stress and Periodic Crises • How is the family affected with this ongoing stress? • How do they react when there is an exacerbation? • How do families endure this? • What are their coping mechanisms?

6. Assisting Family Members in Managing their Feelings 7. Establishing a Support System

Examples of Chronic Illnesses

• Cancer • Neurologic (CP, Epilepsy) • Connective Tissue Diseases (Arthritis) • Asthma • Congenital anomalies (CHD) • Muscular dystrophy • Sickle cell anemia

• Diabetes • HIV • CKD

NEUROLOGIC: Cerebral Palsy Definition

• A group of non – progressive disorders of movement and posture due to non-‐ progressive brain dysfunction

• “Static encephalopathy” • The full extent of motor disability may not be evident until 3

or 4 years of age. • Intellectual, behavioral, and/or sensory difficulties may

accompany, but not a part of the diagnostic criteria. • Prevalence: 1.5 -‐ 2.5/1,000 live births

o Does not reflect the large reduction in neonatal mortality that has taken place over the past 20 years.

• 5-‐15% of surviving low birth weight infants When to Suspect?

• Term infants who are ill in the neonatal period: HIE, low APGAR score( <5), seizures in 1st day of life, continuing neurologic abnormality : 55% risk for chronic motor disability

• Approx 80% -‐ no substantive asphyxia • Presence of malformations and dysmorphic features • About 1/3 of CP in term infants occurred in association with

cortical dysgenesis • Low birth weight infants: low birth weights and abnormally

short gestation are significant risk factors for CP • Preterm infants who are SGA are at special high risk • Congenital malformations located outside of CNS inc the risk

for CP • Chorionitis is a risk factor for CP, regardless of birth weight • Twin births – contribute to 10% of CP in the US • Intrauterine and neonatal infections such as TORCH and

other infections • Maternal conditions such as unusual menstrual period,

thyroid d., estrogen adm have been asso with CP • Hyperbilirubinemia : kernicterus • Motor milestones are the most commonly used features to

clinically monitor CP: developmental quotient for motor skills (MQ)

• MQ = motor milestones / chronologic age x 100% (half correction for prematurity)

• MQ of 50% -‐ 70% is borderline; <50% is associated with significant pathology

• Hand preference prior to the first birthday • Unexplained assymmetry after 3 months • Persistence of primitive reflexes > 6mos:

o prominent fisting o obligate positive support reflex o ATNR o Moro reflex o Hypotonia/ hypertonia o Hypereflexia and ankle clonus

Classification according to area of brain involved: 1. Spastic – most common, motor part of cerebral cortex

• Hemiplegia: Arm and leg on one side is involved • Diplegia: Both legs weak and spastic, arms less weak.

Associated with premature birth


• Quadriplegia: All extremities are severely involved; prominent bulbar signs

2. Dyskinetic (Extrapyramidal) -‐ basal ganglia affected • Choreoathetoid • Dystonic

3. Ataxic – cerebellum and adjacent brainstem involved 4. Mixed type – large areas of cortex and subcortical area including basal ganglia involved CP – hemiplegic type

• 7 year old boy with left hemiplegia predominating markedly in upper limb.

o Abduction of the arm, flexion at the wrist. o No pincer grasp

CP – diplegic type

• Increased muscle tone in the lower extremities. • When the child is held vertically, the legs extend and assume

a scissored position. • Legs are often internally rotated.

CP – quadriplegic

• Bilateral spasticity predominating in the upper limbs with involvement of bulbar muscles

• Almost always in association with severe subnormality and microcephaly

• Totally dependent ADL CP – dyskinetic

• The general appearance of the child is striking with grimacing face and bizarre contractions at each attempted movement.

• Swallowing difficulties • Drooling

CP – ataxic

• Ataxia affecting both lower and upper limbs with dysmetria and intention tremor.

• Able to walk by 3-‐4 years of age although they may fall frequently.

• Wide-‐based stance Etiologies

• Unknown • Prenatal factors • Perinatal factors • Postnatal factors

DIAGNOSTICS

• Cranial ultrasound • CT scan if suspecting TORCH • MRI – for malformations, i.e. cortical dysplasia • Metabolic work up as needed • EEG

Associated conditions

• Cognitive/behavioral o Mental retardation (65%) o Learning disabilities o ADHD and other behavior problems

• Epilepsy (33%) • GI

o Dysphagia o GE-‐reflux

o Failure to thrive • Orthopedic

o Hip dislocation o Scoliosis o Joint contractures o Arthritis

• Hearing and visual loss • Respiratory problems

o Obstructive sleep apnea Factors affecting prognosis

• Type of cerebral palsy • Degree of delay in meeting milestones • Pathologic reflexes • Degree of associated deficits in intelligence • Emotional adjustment

Treatment

• Physiotherapy o manipulation of or exercising affected muscles can

help contain contractures o Night splints to ankles – stretching of achilles tendon. o Soft splinting – lessens knee contractures o Serial plastering – may restore range of movement o Ankle-‐foot orthoses

• Pharmacologic o Diazepam o Baclofen

§ Oral – not effective § Intrathecal (continuous infusion or boluses)

o Botulinum toxin • Surgery

o Lengthening of muscles (achilles tendon) o Soft tissue surgery around the hips and knee o Correction of dislocations (hip) o Transferring a tendon

• Ex. With an overflexed wrist, the surgeon may transplant a wrist flexor onto the dorsum of the hand to increase extensor.

General principles of treatment

• Define long term objectives not only in terms of motor condition o Cognitive skills o Social skills o Emotional status o Vocational potentials o Availability of family support

• Consider patients’ age and growth and development • Consider alternative approaches: family based and culture

competent Prevention

• Avoiding alcohol and smoking during pregnancy • Preventive work in Rh (-‐) mothers • Regular antenatal care • Immunization program for rubella • Newborn screening

o Early detection of amino acid disorders, hypothyroidism and galactosemia

10 PEDIATRICS2 – LE6 Handout [by: msredlips ]

PULMONARY: CHRONIC COUGH CAUSES OF CHRONIC COUGH

• Allergic Rhinitis • Classic Asthma • Cough-‐variant Asthma • Respiratory Infections, with or without asthma • Immunodeficiency Syndromes • Sinus Infections especially those caused by: Streptococcus

pneumoniae, Moraxella catarrhalis, Nontypable H. influenzae

• Cystic Fibrosis • Ciliary Dyskinesia • Foreign body aspiration • Irritation: Air pollution, tobacco smoke, wood smoke, glue

sniffing, volatile chemicals • Swallowing Dysfunction • Gastroesophageal reflux • Habit cough • Anatomic abnormalities: TEF, Laryngeal cleft, Vocal cord

paralysis, Tracheobronchomalacia • Neurodevelopmental delays leading to frequent aspiration • Pulmonary sequestration • Bronchogenic cyst • Mediastinal tumors • Congestive heart failure • Interstitial lung disease

Differential Diagnosis of Chronic Cough (in descending order of likelihood)

MOST COMMON CAUSES OF CHRONIC COUGH Asthma

• Chronic inflammatory disease of the airway with variable airway obstruction and airway hyperresponsiveness

Clinical Manifestations

• cough • chest tightness • wheezing • Breathlessness • Gurgly chest (halak)

Trigger Factors of Asthma in Various Age Groups

Modified Asthma Predictive Index

DIAGNOSIS OF ASTHMA

In past 12 months, ≥ 4 wheezing episodes (>24h), with at least 1 physician-‐confirmed, PLUS

1 Major Criterion OR 2 Minor Criteria Parent with asthma Wheezing apart from colds Atopic dermatitis Eosinophilia (≥ 4%)

Allergic sensitization Allergic sensitization to to ≥1 aeroallergen* milk, egg, or peanuts


PHARMACOLOGIC APPROACH FOR INITIATING THERAPY Classification of Asthma by Severity Before Initiation of Treatment

Clinical Control of Asthma

• Determine the initial level of control to implement treatment (assess patient impairment)

• Maintain control once treatment has been implemented (assess patient risk)

LEVELS OF ASTHMA CONTROL

Monitoring to maintain control

• Control should be monitored to maintain control and establish lowest step and dose

• After the initial visit: 1-‐3 months and every 3 months thereafter

• After an exacerbation follow-‐up: within 2 weeks to 1 month Allergic Rhinitis

• nasal hypersensitivity symptoms induced by an immunologically mediated (most often IgE-‐dependent) inflammation after the exposure of the nasal mucous membranes to an offending allergen.

• Symptoms o Rhinorrhea o nasal obstruction or blockage o nasal itching o Sneezing, o postnasal drip o Allergic conjunctivitis

• Asthma is found in as many as 15% to 38% of patients with allergic rhinitis

• a symptomatic disorder of the nose induced by an IgE mediated inflammation after allergen exposure of the membranes of the nose

• sneezing, rhinorrhea, nasal pruritus

• PND, throat clearing, lacrimation, allergic shiners, allergic salute, facial grimace

• Comorbidities: asthma

sinusitis otitis media conjunctivitis

Differentials

• Vasomotor rhinitis • NARES • Infectious rhinitis • Rhinitis medicamentosa • CSF rhinorrhea • Foreign body

Classification of allergic rhinitis


IMMUNOLOGY ATOPIC DERMATITIS

• Itch that rash • INFANT PHASE

o birth-‐2yo o cheeks, abdomen, extensor

• CHILDHOOD PHASE o 2-‐12 yo o Flexural

• ADOLESCENT/ADULT PHASE o diffuse lesions with increased scaling & decreased

excoriations (3 major, 3 minor) MAJOR MINOR family hx of atopy xerosis (dry skin) area of distribution nipple eczema relapsing course pityriasis alba pruritus dermatographism keratosis pilaris (chicken skin) food intolerance

palmar hyperlinearity dennie morgan fold

DIFFERENTIALS

• Seborrheic Dermatitis • Diaper Dermatitis • Scabies • Psoriasis • Dishydritic Eczema

Management

• Hydration • Eliminate Triggers • Antihistamines • Emollients • Steroids • UV light • Antibiotics

IMMUNODEFICIENCY

• 8 or more EAR infections within 1 year • 2 or more serious sinus infections within 1 yr • 2 or more mos on antibiotics with little effect • 2 or more pneumonias within 1 year • Failure to thrive • Recurrent deep skin or organ abscess • Persistent thrush in mouth/skin after age 1

• Need for IV antibiotics to clear infections • 2 or more deep seated infxs (meningitis, osteomyelitis,

sepsis, cellulitis) • Family history of immunodeficiency

HIV/AIDS

• HIV is a virus (a retrovirus) o HIV-‐1 – Primarily in US o HIV-‐2 – Primarily in Africa & Asia

• AIDS is a disease o Virus attacks immune system o Utilizes CD4 T-‐cell to reproduce its genome o T-‐cells die leading to increased vulnerability to rare

opportunistic diseases o Complications and death result from these diseases

Epidemiology

Routes of Infection

• Sexual activity involving the exchange of body fluids. • Sharing contaminated needles. • Birth by infected mother.


From HIV Infection to AIDS: Four Stages of Progression • Mild symptoms like those of other diseases (e.g., soar throat,

fever, rash, headache). Lasts 1-‐8 weeks. • Latent period for as long as 10 years with no or few

symptoms. • AIDS related complex – cluster of symptoms (e.g., swollen

glands, loss of appetite, fever, fatigue, night sweats, persistent diarrhea).

• Severe immune impairment – multiple opportunist infections (e.g., lungs, gastrointestinal tract).

AIDS Diagnosis

• “AIDS” diagnosis after development of: o Pneumocystis carinii pneumonia o Kaposi’s sarcoma o CD4 levels < 200/microleter.

• Viral load test: determines level of HIV in body. AIDS Treatment Options

• Hope for eventual vaccine • Anti-‐retroviral drugs (Nevirapine, Zidovudine)

Role of Psychology

• Helping people with HIV o Psychological impact of HIV o Compliance with medical regimes o Palliative care o Increasing family support

• Prevention o Behavioral Measures o Personality/Coping

Psychosocial Impact

• Stigma -‐ Fear, blame & rejection from others. • Concerned about who in their social network they can tell. • Increased anxiety, depression and coping problems

Adverse Psychosocial Factors and AIDS Progression

• Yoichi & Vedhara (2009) • Meta-‐analysis examining psychosocial factors on

immunological and clinical indicators of disease progression o Social isolation, life events, depression, anger,

distress, neuroticism, lifetime traumas, avoidant coping

o Mortality, symptoms, CD4 decline, AIDS stage AIDS Prevention

• Education is not enough – continue high levels of risk behaviors.

• Abstinence is not enough • Need new intervention strategies.

o Condom use o Needle Exchange

• Risk may be linked to certain personality characteristics. ENDOCRINE Diabetes Mellitus Criteria for the diagnosis of diabetes mellitus

• Symptoms (polyuria, polydipsia, weight loss) of diabetes plus 1. RBS≥11.1 mmol/L (200 mg/dl)

or 2. FBS≥7.0 mmol/l (≥126 mg/dl).†

Fasting is defined as no caloric intake for at least 8 h.

or 3. 2-‐hour postload glucose ≥11.1 mmol/l (≥200 mg/dl) during an OGTT.

The test should be performed as described by WHO (86), using a glucose load containing the equivalent of 75 g anhydrous glucose dissolved in water or 1.75 g/kg of body weight to a maximum of 75 g (65).

Pathogenesis of type 1 diabetes

• Individuals have an absolute deficiency of insulin secretion and are prone to ketoacidosis

• Most cases are primarily due to T-‐cell mediated pancreatic islet β-‐cell destruction, which occurs at a variable rate, and becomes clinically symptomatic when approximately 90% of pancreatic beta cells are destroyed.

• Serological markers of an autoimmune pathologic process, including islet cell, GAD, IA-‐2, IA-‐ 2β, or insulin autoantibodies, are present in 85-‐90% of individuals when fasting hyperglycemia is detected.

Management of Diabetes Mellitus

• Diet • Medication

o Insulin injections o Meds to control glucose in other ways

§ Compliance ranges from 67% to 85% o Hypertension

§ 30% to 90% do not take as prescribed

o Cholesterol control § 50% stop taking after 6 months

• Exercise • Stress management

Adherence to Diabetic Regimen

• 80% of patients administer insulin in an unhygienic manner. • 58% administer the wrong dose of insulin. • 77% test or interpret the glucose levels incorrectly. • 75% don’t eat the prescribed foods. • 75% don’t eat with sufficient regularity.

Psychosocial Factors

• Social support: Increases adherence • Self-‐efficacy: Increases self-‐management and optimism • Self-‐image: Adolescents • Stress: Causes less insulin and more glucose production


Congenital Adrenal Hyperplasia • Measured by 17-‐OHP in NBS • Prevalence 1:10,000

Definition of Terms

• A disorder in adrenal steroidogenesis arising from a deficiency in enzymes essential to cortisol, aldosterone and androgen production

Steroidogenesis

21-‐Hydroxylase Deficiency

• >90% of CAH • Required for synthesis of Cortisol & Aldosterone • Types:

o Classical § Salt wasting – most severe § Simple Virilizing

o Non-‐Classical – mildly elevated androgens • Epidemiology: common in Philippines 1:10,000 • Genetics: chromosome 6 • Symptoms:

o Progressive wt loss, anorexia o Vomiting, dehydration o Weakness, hypotension o Hypoglycemia, hyponatremia, hyperkalemia

• May appear 2 wks age • If untreated: shock, arrythmia, death

Diagnostics

• 11-‐deoxycortisol, deoxycorticosterone: elevated • Plasma renin: suppressed • Aldosterone low • Hypokalemic alkalosis

Treatment

• Hydrocortisone 10-‐15mg/BSA/day • Fludrocortisone 0.1mg per day

Congenital Hypothyroidism

• Positive NBS if TSH is elevated • Confirmatory: Elevated TSH and Low FT4 • Treatment: Levothyroxine 10-‐15mcg/k/day

• 85% of congenital hypothyroidism is due to thyroid dysgenesis

• 10-‐15% due to dyshormonogenesis

Metabolic Syndrome

ONCOLOGY Leukemia

• Etiology is unknown and multifactorial • Genetics and environmental factors play important roles • Affects about 40/1M children under 15 yrs • ALL (acute lymphoblastic leukemia) accounts for ~ 75% of

cases • CML (chronic myelogenous leukemia) ~ <5% • Signs and symptoms

o Bone marrow failure (anemia, neutropenia, thrombocytopenia)

o Specific tissue infiltration (lymph nodes, liver, spleen, brain, skin, bone)

• Presents with: pallor, petechiae, ecchymose,lethargy, bone or joint pains

• PE o Lymphadenopathy o Hepatosplenomegaly o CNS involvement

• Laboratory and Imaging Studies o CBC, PBS o Bone marrow aspirate o CXR o Ctogenetic analysis

• Treatment o Chemotherapy o Cell transplantation


Tumors AGE is important

Most frequently encountered malignant abdominal masses

• Neuroblastoma • Wilms’ tumor • Hepatoblastoma • Lymphoma

Neuroblastoma

• Occurs anywhere along sympathetic chain or adrenal medulla o But abdominal in 65% of cases

• Most common extracranial tumor in infants • 36% < 1 year old • 75% < 4 years old • Metastatic at dx: 75%

S/Sx highly associated with Neuroblastoma

• Anorexia, wt loss, pallor, abd pain, irritability, weakness • Exophthalmos • Periorbital hemorrhage(raccoon eyes) • Horner’s syndrome • Meiosis, ptosis, enophthalmos, anhydrosis (2 cervical

sympathetic involvement) • Massive hepatomegaly • Constipation, abdominal pan • Localized back pain, weakness • Scoliosis, bladder dysfunction • Palpable nontender subQ nodules (neonatal) • Elevated urine VMA

Wilms’ Tumor

• Arises from embryonic renal precursor cells • Most common pediatric malignancy of the kidney • Peak age at diagnosis: 2-‐3 years (80% diagnosed before 5

years of age) • Rare in infants • Asymptomatic mass in flank • 25% with associated systemic S/Sx

o Malaise o Pain o Hematuria (usually microscopic) o Hypertension (inc renin) o Hemorrhage into tumor (10% of cases)

HEPATOBLASTOMA

• Most common liver tumor of childhood (liver tumors = only 1-‐2% of childhood cancers)

• Mean age at dx – 1 year (80% diagnosed before 3 yrs old) • Advanced disease at presentation – 40%

Signs and Symptoms

• Asymptomatic abdominal mass • Anorexia, pain, weight loss (15%) • Jaundice – rare

Laboratory Findings

• Thrombocytosis (as high as 1,500,000) • AFP – elevated in almost all

Lymphoma

• Diffuse aggressive malignancy in children • Can present at 1-‐5 years old, but more common in older

children and adolescents • 60% NHL; 40% Hodgkin’s • 1/3 of NHL present with abdominal disease

Lymphoma s/sx

• Abdominal pain • Vomiting • Diarrhea • Abdominal distention • Intussusception • Peritonitis • Ascites • Intussusception in child > 1 year old – strong warning to look

for lymphoma Physical Findings associated with abdominal masses

• Many asymptomatic and diagnosed accidentally à take a few seconds to palpate abdomen in all patients

• Location , size, consistency of mass • Bruit , ascites, distended superficial abdominal veins

Routine lab and imaging studies needed

• CBC • Coagulation studies • Urinalysis • Tumor markers • Plain abdominal x-‐ray / UTZ / CT scan

CARDIAC Approach to Heart Diseases in Children Congenital vs. Acquired?

• History • Physical Examination • Chest X-‐Ray • EKG • 2DEchocardiography

Maternal History Infections: 1st trimester of pregnancy

1. German measles -‐ Congenital Rubella Syndrome; PS; PDA 2. Cytomegalovirus -‐ teratogenic

Herpes -‐ myocarditis – last trimester Coxsackie B Illness

1. DM -‐ cardiomyopathy (HOCM) -‐ structural (VSD, TGA,PDA)

2. SLE -‐ Heart Blocks 3. CHD -‐ 15% incidence of CHD

(vs 1% general pop.)


Medications 1. Amphetamines (uppers) – VSD, PDA, ASD, TGA 2. Anticonvulsants

a) Diphenylhydantoins – PS, AS, COA b) Trimethadione – TGA, TOF, HLHS

3. Progesterone/Estrogen – VSD, TGA, TOF Alcohol -‐ Fetal Alcohol Syndrome -‐ VSD, PDA, ASD, TOF Past History

1. Cyanosis including “spells” § Cyanosis -‐ deep & fast breathing

vs Breath holding spell -‐ holding breath

§ Cyanosis – birth (or 2 wks of life) emergency 2. CHF 3. Weight gain/feeding 4. Heart murmur 5. Frequent Respiratory Infections (lower)

• Weight affected more than the height • Weight severely affected – dysmorphic conditions • Others:

o Chest pains o Joint swellings o Neurologic symptoms

Family History

• Incidence in general population – 8 to 12/1000 live births • Recurrence Risk related to recurrence risk of the syndrome or

H.D. • One child affected – risk recurrence in sibling – 3% (VSD)

– 2.5 % (TOF)

Inspection & Palpation: Extremities

• Hands & feet o Cyanosis : clubbing of fingers & toes

Inspection & Observation: Genetic Abnormalities

Inspection & Palpation: Chest • Apex beat – most lateral cardiac impulse • Point of maximal impulse

Inspection & Palpation: Abdomen

• Distension – ascites • Pulsatile abdominal aorta – aortic run-‐off • Liver

o Infants : soft palpable 2 –3 cm BRCM 1 year old : 2 cm & 4 – 5 years old : 1 cm

o Hepatomegaly : hallmark of systemic venous congestion in infants

o Pulsatile liver : TR or inc. RA pressure Palpation: Pulses

• Rate • Regularity • Quality :

o Rate of rise o Pulse volume

§ Fast & brisk : VSD, MR § Fast & large: PDA, AR, severe anemia

Simultaneous palpation of peripheral pulses – delay in lower extremities is suggestive of coarctation HEART MURMURS Location


QUESTION #1: Does the patient have a MURMUR? NO The absence of a murmur does not rule out heart disease in the presence of other cardiac signs and symptoms. Correlate with clinical history and ask the other questions below. YES PATHOLOGIC vs. PHYSIOLOGIC (innocent/functional) TIMING PATHOLOGIC PHYSIOLOGIC SYSTOLE Ejection All >Gr.3/6 Gr.3/6 or less <Gr.3/6 but with Normal heart sounds click or abnormal No clicks heart sounds Regurgitant All regurgitant and Never late systolic DIASTOLIC All diastolic Never CONTINUOUS All except venous Only venous hum hum Does the patient have congenital heart disease? Cyanotic Congenital Heart Disease

• Truncus Arteriosus • Transposition of Great Vessels • Tricuspid Atresia • Tetralogy of Fallot • Total Anomalous Pulmonary Venous Return • Ebstein’s Disease • Eisenmenger’s Syndrome

COMMON CXR OF CHD Ventricular Septal Defect

• Increased vascularity • Normal or enlarged cardiac size • Chamber prominence:

o either or both ventricles o left atrium

• Enlarged main and central pulmonary arteries

• Normal or small aorta Tetralogy of Fallot

• Decreased vascularity • Normal or enlarged cardiac size • right ventricular prominence • Concave main pulmonary artery

segment • Prominent aorta • BOOT SHAPED HEART • right aortic arch (in 20-‐25%)

Cyanotic Congenital Heart Disease Transposition of Great Arteries: CXR

Egg on its side

RV cardiomegaly; narrow pedicle; hypervascular markings

Total Anomalous Pulmonary Venous Return • Increased vascularity • Cardiomegaly • Chamber prominence:

o right atrium o right ventricle

• Enlarged systemic vein into which drainage occurs

• Type I (Supracardiac) o left-‐sided vertical vein connects pulmonary venous

confluence to the left innominate vein, right SVC or azygos vein

o “SNOWMAN APPEARANCE” EBSTEIN’S ANOMALY

Cardiomegaly, decrease pulmonary vascular markings;

WALL TO WALL HEART Rheumatic Heart Disease

• Chronic valvar heart disease as a sequelae of rheumatic fever & its recurrences

• Most common cause of acquired heart disease in children & young adults worldwide

• Most common cause of heart failure among school-‐aged children & young adults

RENAL Chronic Kidney Disease

• Congenital and obstructive abnormalities are the most common causes

• Present between birth to 10 yo (FSGS, chronic GN) • Signs and Symptoms

o Growth failure o Renal osteodystrophy o Anemia o Hormonal abnormallities

• Treatment o Multidisciplinary o Symptomatic treatment – eg. Acidosis – NaHC03; ROD –

phosphate binders; anemia – erythropoietin o dialysis o Optimal: renal transplantation

-‐-‐END J-‐-‐

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