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Clinical Features

• edema

often first sign;detectable when fluid retention exceeds 3-5% of body weight

starts periorbital and often pretibial -> edematous areas are white,soft, and pitting

gravity dependent:periorbital edema

*

and pretibial edema t over the day

anasarca may develop (ie.marked periorbital and peripheral edema, ascites, pleural effusions,

scrotal/labial edema)

• non-specific symptomssuch asirritability, malaise,fatigue, anorexia, or diarrhea

• decrease in effective circulating volume (e.g.tachycardia, HTN,oliguria, etc.)

• foamy urine is a possible sign of proteinuria

Daily protein excretion can be estimated

from a random urine protein

'

creatinine

ratio

Investigations

• urine

urine dipstick (3to 4+ proteinuria, microscopic hematuria) and microscopy (oval fat bodies,

hyaline casts)

first morning urine protein/creatinine ratio (>300 mg/mmol)

• blood work

diagnostic: hypoalbuminemia (<25 g/L), hyperlipidemia/hypercholesterolemia (total cholesterol

>5 mmol/L)

• secondary:electrolytes (hypocalcemia, hyperkalemia, hyponatremia), renal function (t BUN and

Cr), coagulation profile (

*

PTT)

appropriate investigations to rule out secondary causes:CBC, blood smear,C3/C4,ANA, hepatitis

B/C titres, ASOT, HIV serology, etc.

• consider renal biopsy if:HTN,gross hematuria,

* renal function,low serum C3/C4, no response

to steroids after 4 wk of therapy,frequent relapses (>2 in 6 mo), presentation before first yr of life

(high likelihood of congenital nephrotic syndrome),presentation >12 yr (rule out more serious renal

pathology than MCD)

Management

• MCD:oral prednisone 2 mg/kg/d (or equivalent) for up to 12 wk -> varicella statusshould be known

before starting

• consider cytotoxic agents,immunomodulators,or high-dose pulse corticosteroid if steroid resistant

• symptomatic

edema:salt and fluid restriction, possibly diuretic (avoid ifsignificant intravascular depletion);

furosemide + albumin for anasarca

hyperlipidemia:generally resolves with remission;limit dietary fat intake;consider statin therapy

if persistently nephrotic

• hypoalbuminemia:IV albumin and furosemide not routinely given;considerif refractory edema

abnormal BP: control BP;fluid resuscitation ifsevere intravascular depletion; ACHI or AKBs for

persistent HIN

• diet: no added salt;monitor caloric intake and supplement with Ca -+ and vitamin D if on

corticosteroids

• daily weights and BP to assess therapeutic progress

• secondary infections:treat with appropriate antimicrobials;antibiotic prophylaxis not recommended;

pneumococcal vaccine at diagnosis and varicella vaccine after remission;varicella Ig + acyclovir if

exposed while on corticosteroids

• secondary hypercoagulability:mobilize, avoid hemoconcentration due to hypovolemia, prompt sepsis

treatment; heparin if thrombi occur

Prognosis

• generally good:80% of children respond to corticosteroids

• up to 2/3 experience relapse,often multiple times;sustained remission with normal kidney function

usually by adolescence

• complications: t risk of infections (spontaneous peritonitis,pneumonia/empyema, cellulitis,sepsis);

hypercoagulability due to decreased intravascular volume and antithrombin 111 depletion (PH, renal

vein thrombosis); intravascular volume depletion,leading to hypotension,shock,renal failure;

anasarca;adverse effects on growth;drug side effects

Side Effects of Long-Term Steroid Use

GOOD CUSHINGS

Growth Impairment

Obesity

Osteoporosis

Dorsal hump

Changes in behaviour

Ulcers

Striae

Hypertension

Immunosuppression:infection

Need to eat

Glucose elevation

Salt and water retention

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P86 Paediatrics Toronto Xot« 2023

Hypertension in Childhood

Definition

• HTN:sBP and/or dBP >95th percentile forsex.age,and height on >3 occasions

• elevated BP (formerly pre-HTN):sBP and/or dBP >90th percentile but <95th percentile or BP >120/80

irrespective of age,sex, and height

Table 41.95th Percentile Blood Pressures (mmHg)

Female Male

Age (Yr) 50th Percentile for

Height

75th Percentile for

Height

50th Percentile for

Height

75th Percentile for

Height

1 103/60 10461 103/55 10456

6 111/72 112/73 111/71 112/72

122/ 78 124.79 124.78

128 82

121/78

135/85

12

17 127/81 13786

Ftynn JT.Kaetbcr DC.Sahef-Smitll CM. et al.Clinical practice guideline torscreerirg and management of high blood pressure in children and

adotescerts.Pediatrics 2017:140(3|:e20171904

Epidemiology

• prevalence:3-5% for HTN, 7-10% for elevated BP;M>F

Etiology

• primary HTN

diagnosis of exclusion

• most common in older children (>10 yr),especially if positive family history,overweight,and

only mild HTN

• responsible for

-90% of cases of HTN in adolescents, rarely in young children

• secondary HTN

identifiable cause of HTN (most likely etiology depends on age)

• responsible for majority of childhood HTN

• always consider white coat HTN for all ages

Table 42.Etiology of HTN by Age Group

System 1mo-6 yr 7 Neonates -12 yr >13 yr

Endoainopathies'

Essential hypertension

Endocrine Metabolic CAH Endoainopathies'

Essential hypertension

Wilms'tumour(t renin)

Neuroblastoma

|t catecholamines)

Congenitalrenal disease Renal parenchymal

Renal artery thrombosis drsease

Coarctation of the aorta Coarctation of theaorta

Renal artery thrombosis RAS

Renal parenchymaldisease Renal parenchymal

disease

Renovascular disease

Renal

Renovascular

abnormalities

Corticosteroids

Vascular

Corticosteroids

Cyclosporine and

tacrolimus

Drugs Corticosteroids

OCP 0CP

Cyclosporine and

tacrolimus

Cyclosporine and

tacrolimus

Recreational drugs

(amphetamines,cocaine.

etc)

*Nat=:r-sy indi.de hyperthyroidism. hyperparathyroidism.Cushing'

ssyrdrone. primary hyperaldosteronisnu’Conn'

ssyndrome,

pheochronocytoma

Risk Factors

• primary HTN:obesity, male sex, African ethnicity, family history of HTN, LBW

• secondary HTN: prepubertal age, no family history of HTN, history of renal disease,family history of

autoimmune disease

Clinical Features

• often asymptomatic, but can include ITT, fatigue, epistaxis

• symptoms of hypertensive emergency

• neurologic:headache,seizures,focal complaints, change in mental status, visual disturbances

cardiovascular:symptoms of Ml or heart failure (chest pain, palpitations, cough,SOB)

• symptoms ofsecondary HTN:guided by etiology; ask about medications and recreational drugs

(current and past)

Signs of Secondary HTN

• Edema (renal parenchymal disease)

• Abdominal or renal bruit (RAS)

• Differential 4 limb BP/tSmaished

femoral pulses(coarctation)

• Abdominal mass(Wilms'

,

neuroblastoma)

• Goitre/skin changes

(hyperthyroidism)

• Ambiguous genitalia (CAH)

Paediatric BP Calculation

sBP- age x 2 + 90

dBP- 2/3x sBP

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Investigations

• physical exam: upper and lower limb BP with correct cuff size, BM1,full neurologic exam,

ophthalmoscopy, precordial exam, peripheral pulses, perfusion status

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P87 Paediatrics Toronto Notes 2023

•laboratory

• BUN, creatinine, electrolytes, urinalysis, fasting lipid profile

obese patients: HbAlc*

AST, ALT

• further investigations based on history and physical

•imaging: renal ultrasound (with doppler), echo (coarctation, LVH )

•24 h ambulatory blood pressure monitoring (if assessing white coat HTN)

Management

•treat underlying cause

•non-pharmacologic: modify concurrent cardiovascular risk factors (e.g. weight reduction, exercise,

salt restriction,smoking and drug cessation)

•pharmacologic:gradual lowering of BP using thiazide diuretics; no antihypertensives have been

formally studied in children; if hypertensive emergency use hydralazine,labetalol,sodium

nitroprusside

•management of end-organ damage (e.g.retinopathy, LVH)

•consider referral to specialist

Prognosis

•end-organ damage (similar to adults) including LVH,CH1

:

, cerebrovascular insults, renal disease,

retinopathy

Neurology

Cerebral Palsy

Definition

• non-progressive central motor impairment syndrome due to CNS anomaly or neural injury at the

prenatal, perinatal, or postnatalstage

• incidence:1.5-2.5 in 1000 live births(industrialized nations)

• life expectancy is dependent on the degree of mobility and intellectual impairment, not on severity of

CNS lesion

Etiology

• no known cause identified in 1/3of cases

• prenatal causes:TORCH infections, maternal disorders (e.g. epilepsy), congenital CNS anomaly

• perinatal causes:prematurity, LBNV, ischemic stroke

• postnatal causes:infections (e.g. meningitis), asphyxia,1VH, trauma,severe jaundice

Clinical Features

• general signs:delay in motor milestones,developmental delay, learning disabilities, visual/hearing

impairment,seizures, microcephaly, uncoordinated swallow (aspiration)

Table 43. Types of Cerebral Palsy

Type Characteristics Area of Brain Involved

Spastic (70-80%) Truncal hypotonia in first yr

Increased lone, increased reflexes,clonus

Can ailed one limb (monoplegia),one side ol body cerebral artery stroke

(hemiplegia),both legs (diplegia), or both arms and Diplegia associated with periventricular leukomalacia

legs (quadriplegia) in prematurebabies

Ouadriplegia associated with HIE (asphyxia),higher

incidence of intellectual disability

UMN ol pyramidal trad

Hemiplegia most commonly caused bymiddle

Athetoid/Dyskinetic (10-15%) Athetosis tchorea or hypotonia

Can involve face,tongue(resultsin dysarthria)

Poor coordination,poor balance (wide based gait) Cerebellum

Can have intention tremor

More thanone ol the above motor patterns

Basal ganglia (may be associatedwithkemiderus)

Ataxic (<5%)

Mixed (10-15%) Some combinationof the above

Investigations

• neuroimaging (MR1), may include metabolic screen,chromosome studies,serology, EMG, EEG (if

seizures), ophthalmology assessment, audiology assessment

Management

• maximize potential through multidisciplinary services (e.g. primary care physician, OT, FT, SLF,

school supports, etc.)

• orthopaedic and/or neurosurgical management (e.g. dislocations, contractures, rhizotomy)

• management of symptoms:spasticity (baclofen, Botox*), constipation (stool softeners)

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P88 Paediatrics Toronto Notes 2023

Febrile Seizures

Epidemiology

• most common cause of seizuresin children (3-5% of children)

• M>F;age 6 mo-6 vr

Clinical Features

• otherwise well, neurotypical child

• fever often with associated illness (source of fever),family history, past history ofsimple febrile

seizures

• no evidence ofCNS infection/inflammation before or afterseizure;no historyof non-febrile seizures

Table 44.Comparison of Typical and Atypical Febrile Seizures

Simple/Typical (70-80%) Complex/Atypical (20 30%)

All of the following:

Duration <15 rain (95% <5rain)

Generalized tonk-donk

No recurrence in 24 h period

No neurological impairment or developmental delay before or after

seizure

Very smallrisk of developing epilepsy (2% vs.1% in general

population)

At least one of the following:

Duration >15min

Focal onset or focal features duringseizure

Recurrentseizures(>1in 24 h period)

Previous neurological impairment or neurological defidtafterseizure

Increased risk of developing epilepsy

Investigations

• history: determine focus of fever, description ofseizure, medications, trauma history, developmental

history, FMHx

• physical exam:LOC,signs of meningitis, neurological exam, head circumference,focus of infection

• septic workup including LP ifsuspecting meningitis (strongly consider if child <12 mo;consider if

child is 12-18 mo;only if meningeal signs present in child >18 mo)

• if typical febrile seizure,investigations only for determining focus of fever

• EEG/CT/MRI brain not warranted unless atypical febrile seizure or abnormal neurologic findings

Management

• counsel and reassure patient and parents

• febrile seizures do not cause brain damage

• very’small risk of developing epilepsy in simple febrile seizures

33% chanceof recurrence (mostly within 1 yr of first seizure and in children <1 yr)

• antipyretics and fluidsfor comfort (though neither preventseizure)

• no prophylaxis with antiepileptic drugs

• if high-risk for recurrent or prolonged seizures, have rectal orsublingual lorazepam at home

• treat underlying cause of fever

Causes of Hypotonia that Respond to

Rapid Treatment

H4I2SAD

Hypokalemia

Hypermagnesemia

Hypoglycemia

Hydrocephalus

Infection

ICH

Seizure

Acidemia

Drugs/toxins

Hypotonia

Definition

• decreased resistance to passive movements- “floppy baby"

Differential Diagnosis

• central:genetic (DS, Prader-Willi,Fragile X syndrome), metabolic (hypoglycemia, kernicterus),

perinatal problems (HIE,ICH),endocrine (hypothyroidism, hypopituitarism),systemic illness

(TORCH infection,sepsis,dehydration),CNS malformations,dysmorphic sy ndromes

• peripheral:motor neuron (spinal muscular atrophy, polio), peripheral nerve (Charcot-Marie-Tooth

syndrome), neuromuscular junction (myasthenia gravis), muscle fibre (mitochondrial myopathy,

muscular dystrophy,myotonic dystrophy)

Clinical Features

• history:GA,onset/progression,family history of neuromuscular abnormalities,obstetrical history,

birth trauma

• physical exam:

general:dysmorphic features,weight,length, head circumference

MSK:postural movements including traction response, axillary suspension, ventral suspension

(see Figure 17)

neurological:spontaneous posture, muscle bulk, presence of fasriculations

• LMN lesion (60-80%): increased deep tendon reflexes and clonus;floppy and strong; •

Babinski; neonatal reflexes present

• LMN lesion (15-30%); lack of deep tendon reflexes;floppy and weak; •Babinski; neonatal

reflexes absent

Traction response

w

Axillary suspension

1

i

2

;

i

:

J

+

Ventral suspension

V

Figure17.Hypotonia

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P89 Paediatrics Toronto Notes 2023

Investigations

• rule outsystemic disorders (e.g.electrolytes, ABG, blood glucose, CK, and serum/urine investigations

for multiple etiologies including mitochondrial causes)

• neuroimaging:MR1/MRA when indicated

• EMG, muscle biopsy/

• chromosome analysis, genetic testing,metabolic testing, neuromuscular testing,subspecialty

consultations

NCS

Management

• depends on etiology:some treatments available for specific diagnosis

• counsel parents on prognosis and genetic implications

• refer patients for specialized care including:rehabilitation, OT,PT, assess feeding ability

Neurocutaneous Syndromes

Definition

• characterized by skin colour changes and tendency to form tumours of the CNS, PNS, viscera, and skin

In neurocutaneoussyndromes, the

younger the child at presentation,

the more likely they are to develop

intellectual disability NEUROFIBROMATOSIS TYPE I (VON RECKLINGHAUSEN DISEASE)

• autosomal dominant hut 50% are the result of new mutations

• incidence I in 3000, mutation in NTI gene on 17ql1.2 (codes for neurofibromin protein)

• learning disorders, abnormal speech development, and seizures are common

• diagnosis of Nl'

-l requires 2 or more of:

£.

-6 cafc-au-lait spots(>5 mm if prepubertal, >1.5 cm if postpubertal)

£2 neurofibromas of any type or one plexiform neurofibroma

S2 Lisch nodules (hamartomas of the iris)

optic glioma

freckling in the axillary or inguinal region

a distinctive bony lesion (e.g.sphenoid dysplasia, cortical thinning of long bones)

• a first degree relative with confirmed Nl'

-l

• management involves treatment of disease manifestations as they occur, as well as genetic

counselling,OT,PT, and speech therapy as needed

NEUROFIBROMATOSIS TYPE II

• autosomal dominant hut >50% are the result of new mutations

• incidence 1 in 33000, mutation in Nl:

2 gene on chromosome 22 (codes for merlin protein)

• characterized by predisposition to form intracranial,spinal tumours

• diagnosed when (a) bilateral vestibularschwannomas are found, OR ( b) patient has a first-degree

relative with NF-2 AND EITHER unilateral vestibular schwannoma OR any two of the following:

meningioma, glioma,schwannoma, neurofibroma, posterior subcapsular lenticular opacities

• treatment consists of monitoring for tumour development and surgery

Recurrent Headache

• see Neurology. N46

Differential Diagnosis

• primary headache: tension, migraine, cluster

• secondary headache:see Neurology, N47

• anxiety or life stress (e.g. recent move,bullying, parents’divorce, domestic abuse)

General Assessment

• if unremarkable history and physical exam,most likely diagnosis is migraine or tension headache

• CT or MR1 if red flags present

• inquire about level of disability,academic performance, after-school activities

Headache - Red Flags

. First and worst headache of their life

• Sudden onset

. Focal neurological deficits

• Constitutional symptoms

• Worse in morning

• Worse with bending over, coughing,

straining

• Change in LOC

• Sudden mood changes

• Pain thatwakes patient

• Fatigue

. Affecting school attendance

Seizure Disorders

• see Neurology, N18

Differential Diagnosis of Seizures in Children

• benign febrile seizure

CNS:infection,tumour,HIE, trauma,hemorrhage

• metabolic:hypoglycemia, hypocalcemia, hyponatremia

• idiopathic epilepsy and epileptic syndromes

• others:neurocutaneoussyndromes, AVM,drug ingestions/withdrawal

• seizure mimics

Heart problems,such aslong

OT syndrome and hypertrophic

cardiomyopathy, are often misdiagnosed

as epilepsy.Include cardiac causes of

syncope in your differential diagnosis,

particularly when the episodes occur

during physical activity

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Investigations

• lab tests:CBC, electrolytes, calcium, magnesium,glucose, phosphate, + /- ABG,lactate, ammonia

• toxicology screen

. BEG

• CT/MRl if indicated (focal neurological deficit or has not returned to baseline several hours after

seizure)

• consider LP if first-time non-febrile seizure (not indicated for determining recurrence risk of benign

febrile seizures,seizure type,or epileptic syndrome)

Seizure Mimics

• Benign paroxysmal vertigo

. Breath holding

• Hypoglycemia

• Narcolepsy

. Night terror

. Pseudoseizure

• Syncope

. TIA

CHILDHOOD EPILEPSY SYNDROMES . Be

Infantile Spasms

• paediatric emergency as can lead to developmental regression in previously well child and therefore

must be identified and investigated early

• brief, repeated symmetric contractions of neck, trunk,and extremities (flexion and extension ) lasting

10-30s

• occur in clusters;often associated with developmental delay;onset 4-8 mo

• 20% unknown etiology (usually good response to treatment);80% due to metabolic or developmental

abnormalities, encephalopathies,or associated with neurocutaneoussyndromes (usually poor

response to treatment)

• can develop into Westsyndrome (infantile spasms,psychomotor developmental arrest, and

hypsarrhythmia) or Lennox-Gastaut (see below)

• typical EEG: hypsarrhythmia (high voltage slow waves,spikes and polyspikes, background

disorganization)

• management:ACTH,vigabatrin,benzodiazepines

Lennox-Gastaut

• characterized by triad of:

1.multiple seizure types

2. diffuse cognitive dysfunction

3.slow generalized spike and slow wave EEG

• onset commonly 3-5yr

• seen with underlying encephalopathy and brain malformations

• management:valproic acid,benzodiazepines, vagal nerve stimulator,and ketogenic diet;however,

response often poor

Juvenile Myoclonic Epilepsy (Janz Syndrome)

• myoclonus particularly in morning;frequently presents as generalized tonic-clonic seizures

• adolescent onset (12-16 yr);autosomal dominant with variable penetrance

• typical EEG:3.5-6 Hz irregularspike and wave, increased with photic stimulation

• management:lifelong treatment (valproic acid);excellent prognosis

Childhood Absence Epilepsy

• multiple daily absence seizureslasting <30 s without postictalstate that may resolve spontaneously or

become generalized in adolescence

• peak age of onset 6-7 yr,E>M,strong genetic predisposition

• typical EEG:3 Hz spike and wave

• management:valproic acid or ethosuximide

Benign Focal Epilepsy of Childhood with Rolandic/Centrotemporal Spikes

• focal motor seizures involving tongue, mouth, face, and upper extremity usually occurring in sleepwake transition states; remains conscious, but aphasic postictallv

• onset peaks at 5-10 yr; 16% of all non-febrile seizures;remitsspontaneously in adolescence without

sequelae

• typical EEG:repetitive spikes in centrotemporal area with normal background

• management:frequent seizures controlled by carbamazepine, no medication if infrequent seizures

Ketogenic Diet and Other Dietary treatments

lor Epilepsy

Cocteane OB Syst Rev 2012:3X 0001903

Purpose: Systematic review el allstodlesof

Ketogenic and related diets.Included the review ol

4 KCIs.( prospective studies,and 5 retrospective

studies.

Population: Adults and children with diagnosed

epilepsy of any type.

Intervention: Ketogenicdiet. control (placebo diet,

any treatment with known antiepileptic properties).

Main Outcome Measure:Seizure control at3,6.12 mo.

tesulls:Studiesshowed a response rate of at least

38-50% seizure reduction at 3m o.This response

wasmaintained for up to a year,(rangeofside

effects were reported.The mostfrequent were

gastrointestinal effects(30%).

Conclusion: The ketogenic diet isa valid option for

people with medically-intractable epilepsy.

General Approach to Treatment

• education for patient and parents including education and precautionsin daily life (e.g. buddy system,

showers instead of baths)

• medication

initiate: treat with drugappropriate to seizure type;often if >2 unprovoked afebrile seizures

within 6-12 mo

optimize:start with one drug and increase dosage until seizures controlled

if no effect,switch over to another before adding a second antiepileptic drug

• continue antiepileptic drug therapy until patient free of seizuresfor >2 yr, then wean over 4-6 mo

• ketogenic diet (high fat diet): used in patients who do not respond to polytherapy or who do not wish

to take medication (valproic acid contraindicated in conjunction with ketogenic diet because may

increase hepatotoxicity)

• legal obligation to report to Ministry ofTransportation if patient wishes to drive

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Generalized and Partial Seizures

• see Neurology.N18

Respirology

Asthma Updated Guidance for Palivizumab Prophylaxis

among Infants and Yonng Children at Increased

Risk of Hospitalization for Respiratory Syncytial

VimsInfection

Pediatrics 20t4;134(2|:415-420

Palivizumab propitylarisisrecommended for the first

yr nlfife for infants born before 29wk gestation,and

preterm infantswith chrnoic long disease of maturity

(born at <32 vrk gestation and requiring >21\ oxygen

for at Ieast 28d after birth|.Such prophylaxis may

he administered in the first yr of life to infants

with hemodynamicaliy significant heart disease,

and a maximum of S monthly IS mg/kg doses may

he administered during the RSV season to infants

requiring it;infants horn during the RSV season may

need fewer doses.Prophylaxis may he considered

in the first yr of life for children with pulmonary

abnormalitiesor neuromuscular disease impairing

the ability to clearsecretionsfrom the upper airway,

and may he considered for children younger than

24 mo whoare profoundly im munocompromised

during the RSV season. Palivizumab prophylaxis

is only recommended in the second year of life for

children who required at least 2D d ofsupplemental

oxygen after birth with ongoing medical intervention

needs.Monthly prophylaxisshould be discontinued

in children experiencing breakthrough RSV

hospitalizations.Insufficient evidence existsto

support the use of prophylaxisfor chddren with cystic

fibrosisorOown’ssyndrome.

Definition

• see Respirology, R7

• inflammatory disorder of the airways characterized by recurrent episodes of reversible small airway

obstruction resulting from airway hyperresponsiveness to endogenous and exogenous stimuli

• in Canada,the lifetime prevalence in childhood is 10-15% and presents most often in early childhood

• associated with other atopic diseasessuch as allergies and/or atopic dermatitis

Clinical Features

• episodic bouts of wheezing, dyspnea, tachypnea, cough (usually at night/early morning, with activity,

or with cold exposure)

• physical exam may reveal hyper-resonant chest, prolonged expiration, wheeze,or‘quiet’/tight chest

when airflow limited

• symptoms may be exacerbated by “triggers”: UR11 (viral or Mycoplasma), weather (e.g.cold exposure,

humidity changes), allergens (e.g. pets), irritants (e.g. cigarette smoke), exercise, emotionalstress,

drugs (e.g. ASA, p-blockers)

Classification

• mild:occasional attacks of wheezing or coughing (<2/wk);symptoms respond quickly to inhaled

bronchodilators alone and seldomly need inhaled corticosteroids

• moderate: more frequent episodes with symptoms persisting and chronic cough; decreased exercise

tolerance;requiresinhaled bronchodilator and inhaled corticosteroids;sometimes needssystemic

corticosteroids

• severe:daily and nocturnalsymptoms;frequent HD visits and hospitalizations; usually needs systemic

corticosteroids

Management

• acute

Or (keep Or saturation >92%) and fluidsif dehydrated

(52-agonists:salbutamol (Ventolin1

) MD1 + spacer (nebulized or IV in very severe episodes with

impending respiratory failure),5 puffs (<20 kg) or 10 puffs (>20 kg) q20 min for first h

ipratropium bromide (Atrovent*) if severe: MD1 i spacer, 4 puffs (<30kg) or 8 puffs (>30kg) ql5-

20 min x 3 doses

steroids:prednisone (1-2 mg/kg/d x5 d) or dexamethasone (0.3 mg/kg/d x5 d or 0.6 mg/kg/d x2

d); in severe disease, use IV steroids

if no response, add magnesium sulphate

• continue to observe;can discharge patient if asymptomatic for 2-4 h after last dose

discharge home with inhaled corticosteroids (12 wk course;e.g. fluticasone)

• chronic

education,emotional support, avoid triggers, develop an “action plan”, exercise program (e.g.

swimming)

monitor respiratory function with peak flow metre (improves self-awareness of status)

reliever therapy:short acting (52-agonists (e.g.salbutamol)

• controller therapy (first line therapy for all children):low dose daily inhaled corticosteroids

• second line therapy for children <12 yr: moderate dose of daily inhaled corticosteroids

second line therapy for children >12 yr:leukotriene receptor antagonist OR long acting p2-

agonist in conjunction with low dose inhaled corticosteroids;leukotriene receptor antagonist

monotherapy may be considered an alternative second line therapy

severe asthma unresponsive to first and second line treatments:injection immunotherapy

aerochamber for children using daily inhaled corticosteroids

• indications for hospitalization

ongoing need for supplemental Oz

persistently increased work of breathing

> (52-agonists are needed more often than q4 h after 4-8 h of conventional treatment

patient deteriorates while on systemic steroids

Canadian Paediatric Asthma

Consensus Guidelines for Assessing

Adequate Control of Asthma

CMAJ 2005;173(6 Suppl):S12-14

• Daytime symptoms <4 d/wk

• Night time symptoms <1night/wk

• Normal physical activity

• Mild and infrequent exacerbations

• No work/school absenteeism

• Need for (3-agonist <4 doses/wk

• FEV1 or peak expiratory flow >90% of

personal best

• Peak expiratory flow diurnal variation

<10-15%

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P92 Paediatrics Toronto Notes 2023

Bronchiolitis

Bronchodilatori tot Bronchiolitis

CoiMane OB Syst Rev 2010;I2:C00012G6

P urposo lo assess the eHectsof bronchodilatori In

infants with acutebronchiolitis

Methods: Melaanatysisof placebo-controlled RCts

tviiuikngbronchodilators lor bronchiolitis. Oxygen

saturation was the main outcome,

Results: 30 Inals with 1992 infants with bronchiolitis

were included. Oxygen saturation did not improve

with bronchodilatois (meandifference|HD|-0.43.

95% Cl 0.92 to 0.0S|.Neither outpatient (11.9%

vs.15.9%, OB 0.75. 95% Cl1.21|nor inpatient (HD

0.06.95% Cl -0.27 to 0.39) reduced hospitalNation

rates.Effectsonoximetryseenin inpatients|MD

-0.62,95% Cl-140 to0.16|were slightly larger

than for outpatients (MO -0.25,95% Cl -0.61to

0.11). No change in averageclinical score was seen

hinpatients (standardized MO -0.14,95% CI-0.41

to0.12).while a statistically significantdecrease

was seen in outpatients |M0 -0.42.95% Cl -0.79 to

-0.06).Adverse events included tachycardia,oxygen

desaturation,and tremors.

Conclusions: Bronchodilatorsdo nut improve oxygen

saturation,rates of hospital admission, duration

of hospitalizations,or durations loresolution of

illness.They ore not consideredeffective in routine

management of bionchiolitis. especially given their

adverse effeds.

Definition

• LRil, usually in children <2 yr, that has wheezing and signs of respiratory distress

Epidemiology

• the most common LRTI in infants, affects 50% of children in first 2 yr of life; peak incidence at 6 mo,

winter or early spring

• increased incidence of asthma later in life

Etiology

• RSV (>50%), parainfluenza, influenza,rhinovirus, adenovirus, M. pneumoniae (rare)

Clinical Features

• prodrome of URTI with cough and/or rhinorrhea, possible fever

• feeding difficulties, irritability

• svheezing, crackles, respiratory distress,tachypnea, tachycardia, retractions, poor air entry;

symptoms often peak at 3-4 d

Investigations

• routine investigations are not required when bronchiolitis is suspected (Choosing Wisely)

• CXR (only in poor response to therapy or atypical disease): air trapping, peribronchial thickening,

atelectasis, increased linear markings

Management

• self-limiting disease with peak symptoms usually lasting 2-3 wk

• mild to moderate distress

supportive; PO or IV hydration, oral/nasal suctioning as needed, antipyretics for fever, regular or

humidified high flow On

• severe distress

• as above ± humidified high flow C)2 or intubation and ventilation as needed

consider paiivizumab (targets l-

'

-glycoprotein of RSV ) as a prophylaxis in high-risk infants:

bronchopulmonary dysplasia,CHD, congenital lung disease, immunodeficient

• bronchodilators, corticosteroids, and antibiotics have no therapeutic value (unless there is secondary

bacterial pneumonia)

• indicationsfor hospitalization

hypoxia:O’

saturation <92% on initial presentation or increasing O2 requirements

signs ofsevere distress(tachypnea >80/min, tachycardia >180/min, grunting, nasal flaring,

marked chest retractions,lethargy) despite several salbutamol masks

past history of chronic lung disease, hemodynamically significant cardiac disease, neuromuscular

problem, immunocompromised

high-risk infants:history of prematurity (<34 svk), weight <4 kg,age <7 wk

significant feeding problems

socioeconomic barriers to improvement (e.g.inadequate care at home)

Children with bronchiolitis do not

icspond (0 salbutamol.Ipratropium

bromide (A trovenl

"

).or steroids

Cystic Fibrosis

(§>

• see Respirologv. R12

Etiology

• I in 3000 live births, mostly White individuals

• autosomal recessive, mutation in CITR gene found on chromosome 7 (Al-508 mutation in 70%, but

>1600 different mutations identified) resulting in a dysfunctional chloride channel on the apical

membrane of cells

• leads to relative dehydration of airway secretions, resulting in impaired mucociliary transport and

airway obstruction

CF Presenting Signs

CF PANCREAS

Chronic cough and wheezing

F : 1

Pancreatic insufficiency (symptoms of

malabsorption such as steatorrhea)

Alkalosis and hypotonic dehydration

Neonatal intestinal obstruction

(meconiumileus)/Nasal polyps

Clubbing of fingors/Chest radiograph

with characteristic changes

Rectal prolapse

Electrolyte elevation in sweat, salty

Clinical Features

• neonatal: meconium ileus, prolonged jaundice, antenatal bowel perforation

• infancy: pancreatic insufficiency with steatorrhea and ITT (despite voracious appetite), anemia,

hypoproteinemia, hypo

• childhood: heat intolerance, wheezing or chronic cough, recurrent chest infections (

-

S. aureus, P.

aeruginosa, H.influenzae),hemoptysis, nasal polyps, distal intestinal obstruction syndrome, rectal

prolapse, clubbing of fingers

• older patients:COPD,infertility (males),decreased fertility'

(female)

natremia

skin

Absence or congenital atresia of vas

deferens

Sputum with S. aureus or P. aeruginosa

(mucoid)

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P93 Paediatrics Toronto Notes 2023

Investigations

• neonatal screening

• sweat chloride test x 2 (>60 mEq/L)

» false positive tests:malnutrition, atopic dermatitis, hypothyroidism, hypoparathyroidism,GSD,

adrenal insufficiency, G6PD, Klinefeltersyndrome, technical issues, autonomic dysfunction,

familial cholestasissyndrome

false negative tests:technical problem with test, malnutrition,skin edema,mineralocorticoids

• Cl'TK gene mutation analysis:genetic screening panels or gene sequencing if clinically suspicious for

rare mutation, useful when sweat chloride lest is equivocal

• disease often detected during newborn genetic screening; positive result requires DNA testing and

subsequent sweat chloride testing

Management

• nutritional counselling:high calorie diet, pancreatic enzyme replacements, fat soluble vitamin

supplements

• management of chest disease:physiotherapy, postural drainage, exercise, bronchodilators, aerosolized

DNase and inhaled hypertonic saline, antibiotics (e.g.cephalosporin, cloxacillin, ciprofloxacin,

inhaled tobramycin depending on sputum C&S), lung transplantation

• genetic counselling

Complications

• respiratory failure, pneumothorax (poor prognostic sign ), cor pulmonale (late), pancreatic fibrosis

with DM, gallstones, cirrhosis with portal HTN, infertility (male), recurrent respiratory infections

• early death (current median survival in Canada is 46.6 yr)

Pneumonia

Etiology

• inflammation of pulmonary tissue,associated with consolidation of alveolarspaces

Clinical Features

• incidence is greatest in first year of life with viral causes being most common in children <5 yr

• fever, cough, tachypnea

• CXK: diffuse,streaky infiltrates bilaterally

• bacterial causes may present with cough, fever, chills, dyspnea, more dramatic CXR changes (e.g.

lobar consolidation, pleural effusion)

Management

• supportive therapy:hydration,antipyretics, humidified O2

Table 45. Common Causes and Treatment of Community-Acquired Pneumonia

Age Bacterial Viral Treatment

Neonates GBS HSV Ampicillin AND gentamicin

f.eoli CMV

listeria

S. omens

S.pneumoniae

Chlamydia trachomatis

H.influemae

Enterovirus

1-3 mo RSV Ampicillin OR ceftriaxone

Azithromycin (il Chlamydia

(

roctomotosuspeded)

Influenza

Human metapneumovirus

Adenovirus

Parainfluenza virus

RSV High dose amoxicillin OR

ampicillin OR ceftriaxone

3mo-S yr S.pneumoniae

i.aureus

S.pyogenes

Influenza

Human metapneumovirus

Adenovirus

Parainfluenza virus

Influenza High dose amoxicillin OR

ampicillin OR ceftriaxone

Azithromycin OR clarithromycin

(iIUycaplasma'

Chlomydophila

pneumoniae suspected)

S. pneumoniae

Hycaptasmo pneumoniae

Chlamydophila pneumoniae

S. aureus

H.influemae

>Syr

Respiratory Distress

r -> APPROACH TO DYSPNEA

• determine if patient issick or notsick; ABCs

• history: onset, previous episodes, precipitating events, associated symptoms, past medical/family

history of respiratory disease

• physical exam: vitals, SpOz, evidence of cyanosis, respiratory, cardiovascular

• investigations:CBC and differential, electrolytes, BUN.Cr, NP swab, ABG,CXK, ECG ( based

clinical findings)

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P91 Paediatrics Toronto Notes 2023

Dyspnea

I

I I ;

Cardiac Other

tlCP

Ascites

Scoliosis

Pulmonary

CHF I

Cardiac tamponade

Pulmonary edema

Pulmonary embolus

£

Lower airway

Bronchiolitis

Pneumonia

Atelectasis

Asthma

Pleura

Pulmonary ellusion

Empyema

Pneumothorax

Upper Airway

Foreign body

Croup

Laryngeal edema

Epiglottitis

Retropharyngeal

abscess

Tracheitis

Figure 18. Approach to dyspnea in childhood

APPROACH TO WHEEZING

• caused by obstruction of airways below thoracic inlet

Differential Diagnosis of Wheezing

• common: asthma (recurrent wheezing episodes, identifiable triggers, typically >6 yr), bronchiolitis

(first episode of wheezing, usually <1 yr),recurrent aspiration (often neurological impairment),

pneumonia (fever, cough, malaise)

• uncommon: foreign body (acute unilateral wheezing and coughing), CF (prolonged wheezing,

unresponsive to therapy), bronchopulmonary dysplasia (often develops after prolonged ventilation in

the newborn)

• rare:CHF, mediastinal mass, bronchiolitis obliterans, tracheobronchial anomalies

APPROACH TO STRIDOR

• caused by obstruction above the thoracic inlet and may also present with hoarseness and suprasternal

retractions

• stridor at rest is an emergency

• differential diagnosis of stridor: croup, bacterial tracheitis, epiglottitis, foreign body aspiration,

subglottic stenosis(congenital or iatrogenic), laryngomalacia/tracheomalacia (collapse of airway

cartilage on inspiration), retropharyngeal abscess

Table 46. Common Upper Respiratory Tract Diseases in Children

Croup

(Laryngotracheitis)

Bacterial Tracheitis Epiglottitis Choanal Atresia

Posterior nasal

aperture(s)

1in 7000 live births

2 in 3 are unilateral

Affected Site Subglottis trachea Supraglottis

Common in children

6-36 mo

Increased incidence in fall

and winter months

Parainfluenza [75%)

Influenza A and 8

Epidemiology flare Decreased incidence due

to Hib vaccination

Common in children 2-6 yr

Usuallyseen in children3

mo to 6 yr F>M

Etiology S.aureus

5.pneumoniae

H.intluemae

n-hemolytic Strep

M. catarrtrolis

toxic appearance

Rapid progression

Cough

8iphasic stridor

No response to

corticosteroids and

nebulized

epinephrine

Clinical diagnosis

Endoscopy:definitive

diagnosis

H.influenzae

S. pneumoniae

p- hemolytic Strep

S. aureus

Oronasal membrane

persists preventing

the nose joining the

oropharynx

RSV

Adenovirus

Unilateral: diagnosed

later inlife,unilateral

discharge or obstruction

Bilateral:diagnosed

during Infancy,noisy

breathing,cyanosis that

worsens with feeds and

improves when crying

Inability topass NG tube

through nates

CTdelinitlve diagnosis

Clinical Presentation Toxicappearance

Rapid progression

4 Os: drooling,dysphagia,

dysphonia,distress

Stridor

Tripod position

No cough

Fever|»39"C|

Clinical diagnosis

Perform physical exam

cautiously to avoid

exacerbating respiratory

distress

Intubation

IV antibiotics

Non-toxic appearance

Barking cough

Stridor

Viral prodrome

(rliinorrhca.

pharyngitis,cough

llow-grade lever)

Hoarseness

Clinical diagnosis

CXR:"steeple sign" bom

subglottic

narrowing

Investigations

Acute (bilateral choanal

atresia):place oral

airway,initiate gavage

feedings

Long-term:relerral to

otolaryngology

Mild to moderate:

corticosteroids,supportive IV antibiotics

Management Intubation

r

care v. J

Severe:corticosteroids,

nebulized epinephrine,

humidified oxygen,

intubation if necessary

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Rheumatology

Growing Pains

Epidemiology

• age 2-12 yr, M=F

Clinical Features

• diagnosis of exclusion

• intermittent,non-articular pain in childhood with normal physical exam findings

• pain at night, often bilateral and limited to the calf,shin, or thigh; typically short-lived

• relieved by heat, massage,mild analgesics

• child is well, asymptomatic during the day, no functional limitations

• possible family history’of growing pains

Management

• lab investigations not necessary if typical presentation; reassurance and supportive management

Juvenile Idiopathic Arthritis

•a heterogenous group of conditions characterized by persistent arthritisin children <16 yr

•diagnosis:arthritisin 1 joint(s);duration S6 wk;onset age <16 yr;exclusion of other causes of

arthritis;classification defined by features/number of joints affected in the first 6 mo of onset

Systemic Arthritis (Still's Disease)

•onset at any age,M=F

•once or twice daily feverspikes(>38.5®C) >2 d/wk with temperature returning rapidly to baseline;

children usually acutely unwell during fever episodes

•extra-articular features:erythematous “salmon-coloured” maculopapular rash,lymphadenopathy,

hepatosplenomegaly,leukocytosis, thrombocytosis, anemia,serositis, pericarditis

•arthritis may occur wk to mo later

•high tSR,CRP, WBC,platelet count

Oligoarticular Arthritis (1-4 joints)

•most common typeof|IA

onset early childhood (<5 yr),F>M

•persistent affects no more than 4 joints during the disease course

•extended:affects more than 4 joints after the first 6 mo

.typically affectslarge joints:knees (most common), ankles, elbows, wrists;hip involvement unusual

•ANA positive -60-80%, RF negative

•screening eye examsfor asymptomatic anterior uveitis (occurs in -30%)

•complications:knee flexion contracture, quadriceps atrophy, leg-length discrepancy, growth

disturbances, uveitis

Polyarticular Arthritis (5 or more joints)

•ANA positive in 50%, uveitisin 10%

•RF negative (more common)

• onset:2-4 yr and 6-12 yr,F>M

• symmetrical involvement of large and small joints of hands and feet,TM|,cervical spine

•RF positive

onset:late childhood/early adolescence, F>M

similar to the aggressive form of adult rheumatoid arthritis and has a similar course progressing

intoadulthood in most cases

severe,rapidly destructive,symmetrical arthritis of large and small joints

may have rheumatoid nodules at pressure points(elbow'

s, knees)

Enthesitis-Related Arthritis

•onset late childhood/adolescence, M>F

•RF negative arthritis and/or enthesitis(inflammation at the site where tendons orligaments attach to

the bone)

•weight bearing joints, especially hip and intertarsal joints, orsacroiliitis

•risk of developing ankylosing spondylitis in adulthood

•asymmetric involvement oflower limb joints, associated with HLA-B27 and development of

symptomatic uveitis/iritis

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Psoriatic Arthritis

• onset:2-4 yr and 9-11 yr, l

;

>M

• arthritis and psoriasis OK arthritis and at least two of:

• dactylitis, nail pitting or other abnormalities, or family history of psoriasis in a 1st degree relative

asymmetric orsymmetric small or large joint involvement (usually knees and small joints in the

hands and feet)

• erythematous,scaly lesions on scalp, post

-auricular area, peri

-umbilicus, or over extensor surfaces of

elbows and knees

Management

• goals of therapy:eliminate inflammation, prevent joint damage, promote normal growth and

development as well as normal function, minimize medication toxicity

• moderate-intensity exercise (aerobic fitness,flexibility and strengthening exercises) to maintain range

of motion (ROM) and muscle strength

• multidisciplinary approach:OT/PT,social work, orthopaedics,ophthalmology, rheumatology

• 1st line drug therapy: NSAIDs, intra-articular corticosteroids

• 2nd line drug therapy: DMARDs (methotrexate,sulfasalazine,leflunomide), corticosteroids(acute

management ofsevere arthritis,systemic symptoms of J1A, topical eye drops for uveitis), biologic

agents (1L-1/1L-6 inhibition for systemic arthritis,TNFantagonist for polyarticular J1A)

Limb Pain

Evaluation of Limb Pain

Table 47. Differential Diagnosis of Limb Pain

Cause <3yr 3-10 yr >10 yr

Trauma

Infectious

Septic arthritis

Osteomyelitis

Inflammatory

Transient synovitis

> x

> « s

X X X

I

JIA X X

Spondyloarthritls x

SLE x

Dermatomyositis x

HSP x

Anatomic/Orthopaedic

Legg-Calve-Perthes disease

Slipped capitalfemoral epiphysis

Osgood-Schlatter disease

Neoplastic

leukemia

Neuroblastoma

Bone tumour

Hematologic

Hemophilia (hemarthrosis)

Sickle cell anemia

X X

X

X

I X X

X X

X

x x x

X X X

Pain Syndromes

Growing pains

Fibromyalgia

Complex regional pain syndrome

X X

x X

X

Must rule out infection, malignancy, and acute orthopaedic conditions

History

• pattern of onset and progression of symptoms (including acuity and chronicity)

• morning stiffness,limp/weight-bearing status, night pain

• joint involvement (type, distribution) ± spine (axial) involvement

• extra-articular manifestations and systemic symptoms

• functional status:activities of daily living

• family history (arthritis, IBD, psoriasis,spondyloarthropathies, uveitis, bleeding disorders,sickle cell

anemia)

• past medical illness, intercurrent infection,travel,sick contact history, joint injury

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