Paediatric

PAEDIATRIC CARDIOVASCULAR & RESPIRATORY RESOURCE

This resource has been created for medical students in Year 3 and beyond. The purpose of this resource is to familiarise yourself with the pathophysiology, clinical features, investigations and management of common paediatric cardiovascular and respiratory presentations.

Cardiovascular

Acyanotic vs Cyanotic conditions & murmurs

Cardiac embryology

VSD

ASD

ASVD & Eisenmenger's syndrome

PDA

Pulmonary Stenosis

Aortic Stenosis

Transposition of the great vessels

Tetralogy of Fallot

Management

Respiratory

Upper Respiratory Infection

Acute Viral Bronchiolitis

Croup

Pharyngitis

Epiglottitis

Pertussis (Whooping Cough)

Foreign Body Aspiration

Cystic Fibrosis

Bacterial Tracheitis

Pneumonia

MCQ

& Paediatrics rotation tips

By Vidhiya Somasundaram and Brenda Ngo MD 2021

PAEDIATRIC CARDIOVASCULAR & RESPIRATORY RESOURCE

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HOW TO USE THIS RESOURCE

This is an interactive resource is used to return back to the contents slide. The contents slide includes hyperlinks to each condition and the MCQ section. will open a new tab with an associated video. will open a new tab with additional information, images or videos. Disclaimer: This resource contains summarised information about Paediatric Cardiovascular and Respiratory conditions. It does not cover all required topics as part of the Bond Medical Curriculum. Please refer to faculty provided resources for more information.

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TIPS TO PREPARE FOR YOUR PAEDIATRICS ROTATION

Make the most out of your outpatient clinics and ED shifts

Outpatient clinics and ED shifts are the perfect opportunity to review your history-taking, examination and handover skills. These settings cater for you to be the doctor and see your own patients. You get to work them up and handover to seniors to get their opinion.

NO PAEDIATRIC HISTORY IS COMPLETE WITHOUT...

Pregnancy history Neonatal history Developmental history Immunisations Social history Medical history

Establish rapport with both the child and their parents

Practice and Experience your developmental milestones!

Get to know the kid! What do they do for fun? Do they have any pets? How is school going? What subjects do they like at school? What is their favourite food? At the start of every consult, try to speak to the child first and get to know them before diving into the “medical” questions.

Print a table of the developmental milestones and keep it handy. It will be a great tool when assessing how kids are tracking in outpatient clinics and on ward-rounds. It will be good practice for OSCEs and written exams. Seeing patients of different ages will help you remember them. Ask the parents how their child communicates and indicates their needs and wants. This is particularly important for children with a developmental disability where parents can feel as if the questions are creating a negative image of their child because you are describing the things their child is unable to do yet.

Learn how to examine children sitting up in their parent’s lap

Make use of the parents as much as possible, whether as a distraction tool or to gain trust with the child. Perhaps listen to the parent’s chest first so you can show children that it doesn’t hurt. Otoscopy and throat exams may seem daunting especially if the child is squirming around. The best advice is to have the parent wrap the child in their arms or hold the child’s head to make the examination easier for you. Just remember, practice makes perfect!

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SOME ADDITIONAL TIPS...

Take motivation from the gaps in your knowledge that you have noticed during the day at placement

Learn to be flexible with your history-taking and physical examinations

There is a lot of new knowledge for paediatrics. For some of you, you may not have had much interaction with kids previously. Write down topics that you are not sure about. Read up and ask questions. Staff working in the paediatrics units are almost always nice and are keen to help.

Kids may not sit still for you to go through your history-taking and physical examinations systematically.For example, a systematic approach to a baby check would be to go from head to toe. But you may need to listen to the chest first before the baby starts crying and then go back to everything else.

Utilise nurses, allied health and all other staff members

Keep some stickers or a fun Toy on you

Doctors may not have enough time to teach you everything you want to know. Often, other staff members can show you how to change nappies, what and how to feed children or tips on how to get along with difficult children

It is a fun and useful way to keep kids busy or distract them when you are trying to examine them or make them feel more comfortable.

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HAVE FUN!!!

Remember that the aim of this rotation is not for you to know everything the consultant does. Have fun and learn to be flexible with children and their parents.

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Congenital Heart Disease

Congenital Heart Disease (CHD) affects 1-2% of all babies born. CHD is looked for by ultrasound from early in pregnancy and then should be screened for with each physical examination that the newborn has from birth through to the first 12 months of life. CHD can be categorised into acyanotic and cyanotic heart disease. Over 70% of CHD are acyanotic whereas only 30% are cyanotic.

Acyanotic Cyanotic

Acyanotic CHD can be subdivided into those that have a Left to Right shunt and those that are obstructive. Left to Right shunt VSD ASD ASVD PDA Obstructive Pulmonary Stenosis Coarctation of the Aorta Aortic Stenosis

Cyanotic CHD include the following conditions: Tetralogy of Fallot Transposition of the Great Vessels Truncus Arteriosus Tricuspid valve abnormalities Total anomalous pulmonary venous connection Other conditions

This resource will cover a select few of these conditions that may be found more commonly in clinical practice. Refer to home page for list of CHD.

Murmurs

Heart murmurs are extra sounds that are heard outside the regular S1 and S2 in the normal cardiac cycle. Many can be physiological or 'innocent' that occur with normal blood flow, requiring no other investigations or management but it is important to rule out any pathological murmurs.

Pathological Murmur Key Features Duration can vary Extra sounds (clicks or gallop) Can radiate Harsh (high amplitude) Diastolic, systolic, continuous Symptomatic Investigations abnormal Associated cardiac abnormalities Examples Aortic Stenosis Bicuspid Aortic Valve Pulmonary Stenosis ASD Mitral Regurgitation Mitral Stenosis VSD Mitral Valve Prolapse Coarctation of AortaPDA

Innocent Murmur Occurs in 25-50% of children, can be worse if child is unwell Key Features Sensitive (murmur varies with positions/respiration) Short duration Single (no other clicks or gallop) Small (non radiating and localised) Soft (low amplitude) Systolic (occurs only during systole) Symptomless Normal investigations Examples Still's murmur: Systolic and musical murmur heard at lower left sternum Pulmonary flow murmur: Ejection murmur heard at lower left sternum Venous hum: Continuous murmur heard in infraclavicular, changing with neck turns, inaudible when supine Carotid bruit: Systemic flow murmur heard at over carotids

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Cardiac Embryology

Cardiovascular system is one of the first systems to develop in the embryo. It becomes active at approximately four weeks of development.

The separation of the four chambers (Right and Left atria, Right and Left ventricles) takes place in the following pictures.

Foetal circulation tract

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Ventricular Septal Defect (VSD)

Failure of the endocardial cushion to fuse appropriately during embryology resulting in a VSD. Most common CHD. Acyanotic CHD (Left to Right shunt).

Pathophysiology

Normal development: Primitive atrioventricular (AV) canal unites the atria and the ventricles. At 4-5 wks of gestation, the superior and inferior portions of the endocardial cushion of the AV canal merge -> creation of AV valves. Failure of this fusion can result in AV canal defects (e.g ASD, VSD). In VSD: There is higher pressure in left ventricle (LV) > right ventricle (RV). O2 blood from LV flows to RV, -> ↑O2 blood in RV, -> ↑O2 blood in pulmonary system. Can lead to pulmonary hypertension and RV +/- right atrium (RA) hypertrophy.

Clinical Features

Risk Factors

Small defects:

• Usually asymptomatic
• Auscultation: Harsh holosytolic murmur

Large defects:

• Physical signs: clubbing, dyspnoea, palpable thrill
• Auscultation: Harsh holosystolic murmur heard over left lower sternal edge with a mid-diastolic rumble, narrow S2

Can lead to:

• Recurrent respiratory infections
• Failure to thrive (e.g poor growth, poor feeding, etc.)
• Heart failure (due to excessive pulmonary blood flow)

• Eisenmenger syndrome

Associated conditions:

• Trisomy 21, 18, 13
• Foetal alcohol syndrome
• Cri du chat syndrome
• Apert syndrome

Intrapartum:

• Maternal infections
• Smoking exposure

Maternal risk factors:

• Diabetes Mellitus
• Obesity

Investigations

Diagnosis based on clinical finding. ECHO (confirmatory)

• Colour flow doppler

ECG (can find signs suggestive of RV hypertrophy)

• Right axis deviation
• P pulmonale
• PR prolongation
• RBBB (RSR pattern in anterior chest leads)

CXR

• Enlarged RA, RV, pulmonary arch depending on size of defect
• ↑pulmonary vascular marking

Osmosis preview - VSD

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Atrio Septal Defect (ASD)

Failure of the endocardial cushion to fuse appropriately during embryology resulting in an ASD. Accounts for 4-5% of CHD. It is the second most common CHD after VSD. Acyanotic CHD (Left -> Right shunt). Different types of ASD: Ostium secundum (60-90%), Ostium primum (5-20%), Sinus venosus (5%).

Pathophysiology

Normal development: Primitive atrioventricular canal unites the atria and the ventricles. 4-5 wks of gestation, the superior and inferior portions of the endocardial cushion of the AV canal merge -> AV valves. Failure to fusion can result in AV canal defects (e.g ASD, VSD). In ASD: There is higher pressure in the left atrium (LA) > right atrium (RA). O2 blood from LA flows to RA, -> ↑O2 blood in RA -> ↑O2 blood in pulmonary system. Can lead to pulmonary hypertension and RA +/- RV hypertrophy.

Clinical Features

Risk Factors

Small defects:

• Asymptomatic
• Auscultation: Systolic ejection murmur

Large defects:

• Physical signs: clubbing, dyspnoea, exertional fatigue, palpable heave
• Auscultation: Systolic ejection murmur over 2nd intercostal space with a wide and split S2

Can lead to:

• Recurrent respiratory infections
• Failure to thrive
• Heart failure (due to excessive pulmonary blood flow)

• Eisenmenger syndrome

Associated conditions:

• Trisomy 21
• Foetal alcohol syndrome
• Holt-Oram syndrome

Intrapartum:

• Maternal infections
• Drug/alcohol exposure

Investigations

Diagnosis based on clinical findingECHO (confirmatory)ECG (can find signs suggestive of RV hypertrophy)

• Right axis deviation
• P pulmonale
• PR prolongation
• RBBB (RSR pattern in anterior chest leads)

CXR

• Enlarged RA, RV, pulmonary arch depending on size of defect
• ↑pulmonary vascular marking

Osmosis preview - ASD

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Atrio-Septal Ventricular Defect (ASVD)

Group of CHD that involve improper development of the atrial and ventricular septa and associated valves. Acyanotic CHD (Left -> Right shunt).

Pathophysiology

Failure in the development of the endocardial cushion which gives rise to the atrial septum, interventricular septum, AV valves and semilunar valves. This can result in various deformities:

• ASD
• VSD
• AV valves abnormalities

Symptoms of ASVD depend on the severity and location of malformation. ASVD is very common in children with Down syndrome and they usually need surgery early in life as they can be haemodynamically unstable. Refer to ASD and VSD pages for more information.

Eisenmenger Syndrome

Reversal of Left -> Right shunt (acyanotic CHD) to Right -> Left shunt (cyanotic CHD).

The left side of heart is more muscular and stonger in order to generate higher pressures required to pump blood to the highly pressurised systemic circulation. In comparison, the right side only needs to pump blood into the low pressurised pulmonary system. With defects (e.g. VSD, ASD), the blood flows down the pressure gradient from Left to Right. However, this abnormal amount of blood flow to the right side leads to maladaptive changes that eventually leads to pulmonary hypertension. Right ventricular pressure eventually > left ventricular pressure -> reversal of blood flow from Right to Left. Onset of cyanosis is noted with shunt reversal as blood is not oxygenated.

Pathophysiology

↑ Right sided pressure and volume overload leads to a multitude of issues. The delicate low pressurised pulmonary system is damaged causing scar tissue development over pulmonary capillaries. Scar tissue does not contribute to oxygenation and also becomes less compliant than normal lung tissue. This leads to a cascade where the pulmonary blood pressure further increases from the increasing scar tissue but also the heart is forced to pump harder to supply the lungs. Due to this ongoing response, Eisenmenger's syndrome is considered irreversible and dangerous.

Khan Academy - Eisenmenger Syndrome

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Patent Ductus Arteriosus (PDA)

Failure of the ductus arteriosus (DA) to close in the 1st few days of life. Left to Right shunt from aorta to pulmonary artery. Accounts for 5-10% of all CHD. Common in most pre-term babies. There are two types of PDA: Originating from a congenital abnormality and those that fail to close in pre-term infants due to repiratory disease.

Pathophysiology

In foetal circulation, the DA allows for the underdeveloped lungs in-utero to be bypassed (normal: Right -> Left shunt) as the pulmonary vascular resistance is high. At birth, the pulmonary vascular resistance ↓, shunt reversal (Left -> Right) and usually the DA closes to become the ligamentum arteriosum. Failure to close the DA at birth → open channel from aorta to pulmonary artery → Left -> Right shunt → overload of pulmonary artery .

Risk Factors

Clinical Features

Low gestation/prematurity [most important!]Lack of antenatal steroidsMaternal exposure to rubella, alcohol, phenytoin, prostaglandin use Respiratory distress syndrome, need for ventilationAssociation with genetic syndromes, e.g. trisomy 21 and DiGeorge

Small PDA:

• Can be asymptomatic
• Auscultation: Continuous heavy murmur

Large PDA:

• Physical Exam: tachycardia, bounding peripheral pulses, wide pulse pressure
• Auscultation: Continuous heavy/machinery murmour heard best in L 2nd intercostal space

Can lead to:

• Failure to thrive
• Heart failure
• Recurrent respiratory infections

Investigations

Diagnosis based on clinical findings ECHO (Diagnostic)

• Left atrial and ventricle enlargement
• Degree of pulmonary artery pressure & shunt volume
• Doppler: aorta to pulmonary artery flow

CXR

• Normal, cardiomegaly, prominent pulmonary artery + aortic knob, ↑pulmonary vasculature

ECG

• Normal, left ventricular hypertrophy

Osmosis preview - PDA

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Pulmonary Stenosis (PS)

Right ventricular outflow tract obstruction (RVOT) due to PS. Acyanotic CHD.

Pathophysiology

Abnormal development of any of the structures below can lead to PS:a. Supravalvar PS - pulmonary artery above valve is narrowed b. Valvular PS - thickening + narrowing of pulmonary valves c. Subvalvar PS - muscle under valve is thickened , narrowing right ventricular outflow d. Branch peripheral PS - pulmonary artery narrowing PS leads to obstruction of blood flow from the RV into the pulmonary system. As a consequence, pressure increases in the RV leading to hypertrophy and reduced pulmonary blood flow. This can occur in isolation or with other conditions, e.g. Tetralogy of Fallot.

Clinical Features

Risk Factors

Sporadic Associated cardiac conditions:

• Tetralogy of Fallot
• Tricuspid Atresia
• Transposition of the great vessels

Other associations:

• Noonan syndrome
• Alagille syndrome
• Willaims-Beuren syndrome
• Congenital rubella syndrome

Symptoms can vary based on the degree of stenosis.

• Asymptomatic

• Can be undiagnosed until later in childhood
• Physical Exam: Cyanosis, palpable thrill, dyspnoea, oedema
• Auscultation: Systolic ejection murmur with a wide, split S2

• Heart failure symptoms

Investigations

Diagnosis based on clinical finding ECHO with doppler (confirmatory) ECG

• Can be normal
• Right axis deviation + other findings of RV hypertrophy

CXR

• Can be normal
• Cardiomegaly
• Prominent pulmonary artery

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Coarctation of Aorta

Narrowing of aortic isthmus (distal aortic arch) situated next to the left subclavian artery and DA.Coarctation can also occur in the abdominal or thoracic aorta (rarely). Acyanotic CHD.

Pathophysiology

Intrauterine ischemia +/- genetic defect leads to narrowing of the aorta as the aortic lumen thickens to compensate for ischemic damage. The narrowing occurs usually distal to the left subclavian artery and proximal to the DA. ↑Blood flow proximal to and ↓ flow distal to the coarctation. Blood vessles proximal to the coarctation develop collaterals due the elevated blood pressure (BP). Compensatory mechanisms: - Myocardial hypertrophy & collateral blood flow (e.g. intercostal vessels, scapular vessels) develop in cases of discrete stenosis to compensate for the LV outflow tract obstruction -> onset of symptoms usually later in childhood. - Hypoperfusion of organs and extremities distal to the stenosis.

Clinical Features

Risk Factors

Newborn:

• Physical exam: Cyanosis in lower extremities , weak femoral pulses (brachio-femoral delay).
• Important to assess for critical coarctation which presents in first few hours of life after PDA closure by feeling femoral pulses.
• Important to assess for critical coarctation which presents in first few hours of life after PDA closure by feeling femoral pulses.

Children

• History and Exam: Chest pain, cold lower extremities, cyanosis , claudication of lower extremity with exercise, ↑BP in upper extremity, ↓BP in lower extremity

• - Auscultation: Crescendo-decrescendo systolic ejection murmur

Unknown Males > Females Genetic association: Turner syndrome

Investigations

Diagnosis based on clinical finding BP: brachio-femoral delay ECHO with doppler (confirmatory)

• Coarctation location & severity
• Any other anomalies e.g VSD, PDA
• ↓SpO2

CXR

• Rib notching , figure 3 sign
• Cardiomegaly
• ↑pulmonary vascular marking

Genetic screen

• Turner syndrome

Osmosis preview - Coarctation

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Aortic Stenosis (AS)

Left ventricular outflow tract obstruction (LVOT) due to AS. Accounts for 6% CHD. Acyanotic CHD.

Pathophysiology

Abnormal development of any of the structures below can lead to AS:a. Supravalvar AS - aorta above valve is narrowed b. Valvular AS - thickening + narrowing of aortic valves c. Subvalvar AS - muscle under valve is thickened , narrowing left ventricular outflow AS causes obstruction of blood flow from the LV. As a result, pressure increases in the LV eventually leading to hypertrophy. AS can be congenital or be an acquired condition later in adulthood.

Risk Factors

Clinical Features

Symptoms can vary based on the degree of stenosis.

• Asymptomatic
• Physical examination: tachypnoea, palpable thrill over aortic area
• Auscultation: Systolic ejection murmur with an ejection click

Can lead to:

• Heart failure during 1st year of birth
• Cardiogenic shock in neonatal period or after closure of PDA

Unknown Males > Females Associated cardiac conditions:

• Bicuspid Aortic Valve
• Parental congenital AS
• Coarctation of Aorta

Investigations

Diagnosis based on clinical finding ECHO with doppler (confirmatory) ECG

• Can be normal
• Left axis deviation + other findings of LV hypertrophy

CXR

• Can be normal
• Cardiomegaly
• Prominent ascending aorta

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Transposition of the great vessels (TGV)

Anatomical reversal of the aorta & pulmonary artery during development. Cyanotic CHD (Right -> Left shunt).

Pathophysiology

Normal development: aorticopulmonary septum rotates 180o and merges with the ventricular septum. This divides the outflow tract into the pulmonary trunk and the aorta. RV supplying blood to the pulmonary trunk. LV supplying blood to the aorta. In TGV, failure of the aorticopulmonary septal rotation results in reversal of the blood supply: RV blood flow -> aorta LV blood flow -> pulmonary trunk Parallel systemic and pulmonary circulations - ↓Oxygenation of systemic circulation.

Risk Factors

Clinical Features

Postnatal cyanosis (dependent on amount of blood mixing between the parallel systems and associated anomalies)

• Cyanosis independent of exertion

Physical examination: Tachypnoea, tachycardia, diaphoresis, poor growth Auscultation: Single, loud S2; often no murmur, systolic murmur at left sternal border if VSD also present

Unknown Diabetic mothers Genetic syndrome association: DiGeorge syndrome Association with: VSD, ventricular outlet obstruction , abnormal valves and coronary arteries

Investigations

Diagnosis based on clinical findings ECHO (confirmatory test)

• Shows aorta arising from RV and pulmonary artery from LV

CXR

• Enlarged heart, 'egg on a string'
• Narrow mediastinum
• ↑pulmonary vasculature

ECG

• Normal

↓SpO2

Osmosis preview - TGV

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Tetralogy of Fallot

Concurrent occurrence of four defects due to an endocardial cushion defect. 1. VSD 2. Overriding Aorta 3. RVOT 4. Right Ventricular Hypertrophy (RVH)Cyanotic CHD (Right -> Left shunt).

Pathophysiology

Endocardial cushion defect results in: VSD: Results in a shared opening for the aorta & pulmonary artery. Aorta has higher pressure so it takes up most of this opening -> overriding aorta. Overriding aorta pushes aside the pulmomary artery -> pulmonary valve stenosis. RVH to compensate in order to push blood through the pulmonary artery. - Superior and anterior deviation of the infundibular septum -> misaligned VSD with an aortic root overriding the defect -> RVOT due to pulmonary infundibular stenosis RVH due to larger VSD and RVOT causing ↑ systemic right ventricular pressure.

Risk Factors

Clinical Features

Mild: Asymptomatic or cyanosis based on RVOT severity Tet Spells: Intermittent hypercyanotic hypoxic episodes, typically at 2-4 months of age Older children: Squatting - kink in the femoral arteries -> ↑systemic vascular resistance to increase pressure at aorta -> ↓ magnitude of Right to Left shunt across VSD Harsh systolic ejection murmur Heart failure symptoms

Sporadic Genetic associations:

• DiGeorge syndrome
• Down syndrome

Maternal risk factors during pregnancy:

• Alcohol exposure
• Diabetes Mellitus
• Phenylketonuria

Investigations

Diagnosis based on clinical findings ECHO (confirmatory test)

• Defects
• RVOT pressure gradient

CXR

• Boot-shaped heart
• ↓ pulmonary vascular marking

ECG

• Right axis deviation, RVH
• Prominent anterior R waves, posterior S waves

↓SpO2

Osmosis preview - TOF

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Management

Acyanotic CHD

ASD, VSD, ASVD Management is based on: Size and persistence of defect and degree of shunting. Chance of spontaneous closure of defect. If closure is indicated, option between surgical or percutaneous transcatheter closure. Supportive medical management for associated heart failure. Majority of small defects close spontaneously by five years of age and if there are no associated symptoms, closure is not indicated. Larger defects may present with pulmonary hypertension, valvar damage, increased Left to Right shunting resulting in heart failure and exercise intolerance. Closure is indicated in these cases. Heart failure is rarely found in childhood and more apparent in persisting defects in adulthood. Supportive medical treatment for heart failure include: Diuretics, inotropic agents, ACE inhibitors

PDA Management is based on: Age and size of patient. Presence of symptoms. Degree of shunting. Long-term benefits of closure (e.g. limits complications) If closure is indicated, medical vs. surgical intervention? Smaller PDAs can be observed and monitored for regular cardiovascular status, pulmonary vascular changes and associated symptoms. Larger PDAs can result in symptomatic Left to Right shunting -> in heart failure. Closure of PDA can be done medically using indomethacin and ibuprofen (prostaglandin synthesis inhibitors) in pre-term infants. This is not indicated in term infants or older infants. Other closure options include percutaneous occlusion and surgical ligation. For those who develop symptoms of heart failure, medical management is indicated with digoxin and furosemide. If this fails, closure is indicated. *Keep PDA open if needed for survival, e.g. critical AS, TGV, Tetralogy of Fallot. AS, PS Management is based on: Neonates with critical AS or PS needing urgent intervention. If stable, choice of intervention is based on severity of AS or PS. Interventions include: Balloon valvuloplasty or surgical. Infants can develop symptoms of heart failure if persisting stenosis and if untreated, medical management is indicated in this case with: Diuretics, Inotropic agents Prostaglandin E1 intravenous infusion is indicated to maintain a PDA in critical AS (allows the RV to support the systemic circulation) and PS (allows support for the pulmonary system with adequate blood supply).

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Management

Acyanotic CHD

Coarctation of aorta Managed through corrective surgery or transcatheter balloon angioplasty intervention. Indications: Critical coarctation. Gradient of coarctation >20mmHg. Radiological support for significant collateral flow. Consequential heart failure. Consequential systemic hypertension. Neonates with critical coarctation are at risk of developing heart failure and death due to PDA closure. Hence PDA is maintained with a Prostaglandin E1 intravenous infusion before surgery and inotropic support is used. Transposition of the great vessels Managed through surgery (arterial switch procedure of the aorta and the pulmonary artery). This surgery needs to take place within 1-2wks of life as there is a 90% mortality rate without surgery. Prostaglandin E1 intravenous infusion is used to prevent PDA closure until surgery as it is vital for oxygenation of the systemic circulation. Tetralogy of Fallot Managed with surgery: within 1st year of life as there is a 50% mortality rate past three years of life without surgery. VSD closure with correct aorta position. RVOT: Enlargement of lumen to reduce PS. Prostaglandin E1 intravenous infusion is used to prevent PDA closure until surgery to maintain oxygenation. If a child displays signs of a Tet spell: Provide O2 Knee to chest position/squat. IV morphine for sedation/fluids/beta-blockers based on severity of symptoms.

Cyanotic CHD

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Upper Respiratory Tract Infection

An upper respiratory tract infection is an acute, self-limiting infection that involves the nasal cavity, paranasal sinuses, pharynx or larynx. It can be caused by a variety of viruses, most commonly rhinovirus, which causes up to 50% of colds in children and adults. The majority of upper respiratory tract infections are transmitted by direct contact or respiratory droplets.

Pathophysiology

Viral Causes

Rhinovirus (30-50%) Coronavirus (10-15%) Coxsackie Virus A (10-15%) Influenza Virus A & B (5-15%) Respiratory Syncytial Virus (5%) Parainfluneza Virus Types 1, 2, 3 & 4 (5%) Adenovirus (<5%)

Cold viruses attach to receptors on epithelial cells in the nasopharynx and enter the cells. The infected cells release cytokines, including IL-8, which attracts polymorphonuclear cells. PMN cells accumulate in the nasal secretions which in turn slows down mucocilliary clearance. Symptoms usually appear 1-2 days after viral inoculation.

Clinical Features (History)

Clinical Features (Physical Exam)

• Ask about recent sick contacts!
• Infants: Fever, nasal discharge, fussiness, difficulty feeding, decreased appetite, difficulty sleeping
• Children: Nasal congestion, rhinorrhoea, cough, low grade fever, headache, and malaise
• In infants and young children, the symptoms of the common cold usually peak on Day 2 – 3 of illness, and gradually improve over 10-14 days.

• Febrile
• No signs of respiratory distress
• Minimal adventitial signs on auscultation

Management

Investigations

Clinical diagnosis - based on history and physical examination findings

• Reassure parents about the self-limiting nature of the condition
• Supportive care
• Maintaining adequate hydrations
• Ingestion of warm fluids
• OTC products for symptomatic relief including antihistamines, decongestants, paracetamol

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Acute Viral Bronchiolitis

Acute viral bronchiolitis is the most common lower respiratory tract infection in infants and young children (<2 years of age). It is an acute inflammatory injury of the bronchioles, most commonly caused by respiratory syncytial virus . It is self-limiting in the majority of cases, however there are a number of children who require intensive care support to assist them through the peak of illness. The main children at greatest risk of requiring intensive care support from an episode of acute bronchiolitis are: 1. Infants < 6 years old who can develop a viraemia and have an apnoea secondary to RSV infection 2. Children who have chronic neonatal lung disease and remain on supplemental oxygen 3. Children with a neurological disorder such as cerebal palsy, who cannot cough properly 4. Children with a congenital heart disease, particular left to righ shunts, where pulmonary oedema in the lungs makes a severe bout of bronchiolitis more likely

Indications for Hospitalisation

Pathophysiology

Viruses, most commonly respiratory syncytial virus (RSV) infect the nasopharynx causing coryza symptoms. A lower respiratory tract infection can begin 1-3 days later spreading to the terminal bronchiolar epithelial cells causing direct damage and inflammation in the bronchioles. Oedema, excessive mucus and sloughed epithelial cells lead to obstruction and atelectasis of the bronchioles.

Reduced or difficulty feeding (<50% of usual intake in 12 hours) or dehydration Moderate to severe respiratory distress

• Nasal flaring
• Intercostal, subcostal or suprasternal retractions
• Respiratory rate > 70 breaths per minute
• Apnoeic episodes
• O2 saturations < 92%

Clinical Features

Investigations

• Presents with fever, cough and respiratory distress
• Often preceded by 1-3 day history of upper respiratory tract symptoms (e.g nasal congestion and/or discharge)

• PES: Tachypnoea, increased work of breathing, widespread inspiratory crackles, or wheeze

• Clinical diagnosis
• Routine CXR is NOT required for children with typical clinical features, however consider if < 4 weeks or severe

Management

Mild bronchiolitis: Manage at home

• Reassure and educate carers
• Maintain adequate hydration
• Relief of nasal congestion/obstruction

Moderate to severe bronchiolitis, provide symptomatic care in hospital including

• Supplemental oxygen if required
• Nasogastric feeds or IV fluids if normal feeding is not possible

Severe bronchiolitis

• May require non-invasive ventilatory support such as high flow oxygen
• May need IPPV if they develop progressive respiratory failure

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Croup

Croup (laryngotracheobronchitis) is a respiratory illness characterised by inspiratory stridor, barking cough and hoarseness as a result of inflammation of the larynx and trachea. The most common pathogen causing croup is parainfluenza virus (Types 1 & 3) (75%) It usually occurs in children from 6 months to 3 years of age. Although croup is usually a mild and self limiting illness, it can lead to significant upper airway obstruction and respiratory distress. In moderate to severe cases, these children can be at risk of complete upper airway obstruction or appear sick enough that these cases can be confused with acute bronchiolitis.

Pathophysiology

Steeple Sign

A viral infection, usually parainfluenza virus, RSV, adenovirus or influenza causes inflammation of the upper airway. This leads to the formation of oedema and infiltration of inflammatory cells. This causes the subglottic airway to narrow leading to inspiratory stridor and increased work of breathing

Note: Although acute viral infections are the most common cause of croup, bacterial and atypical agents have also been identified

Clinical Features (History)

Distinguishing Features

1. Prodromal Phase (1-2 days)

• Rhinitis with nasal discharge and congestion
• Coryza symptoms (low grade fever)

2. Laryngotracheal Inflammation Phase (2-7 days)

• Worse at night, with sudden onset of symptom
• Seal-like barking cough, hoarseness, inspiratory stridor

Features that are helpful in distinguishing croup from other causes include: Fever, barking cough and hoarseness Croup is the cause of stridor with a barking cough! If there is a cough and no drooling, croup is almost always the diagnosis

Management

Investigations

Usually self-limiting, treat at homeKeep child calm and adopt a position of comfort that minimizes airway obstruction For severe croup, arrange immediate transfer to hospital

• Use adrenaline 0.1% (1:1000, 1mg/mL) solution 5mL by inhalation via nebulizer, repeated after 30 minutes if no improvement + prednisolone OR dexamethasone

Admission criteria:

• Persistent stridor (especially at rest).
• Respiratory distress.
• Multiple doses of racemic epinephrine.
• Possibility of alternate diagnosis.

Clinical diagnosis Chest X-ray usually not necessary, however it is indicated if:

• Course is atypical and/or diagnosis is in question
• Child has severe symptoms and does not respond as expected to therapeutic interventions
• Foreign body is suspected
• Recurrent croup

Steeple sign – narrowing of tracheal air column just below the vocal cords

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Pharyngitis

Pharyngitis is the infection of the tonsils and/or the pharynx. It can be caused by pathogens including bacteria: streptococcal pharyngitis and viruses including rhinovirus, adenovirus, coxsackievirus and Epstein-Barr virus. The majority of pharyngitis episodes are due to viral causes which are usually treated conservatively. However, early recognition of streptococcal pharyngitis is important since failure to treat may result in complications. Unfortunately, it can be difficult to distinguish between bacterial and viral causes.

Clinical Features

Pathophysiology

With infectious pharyngitis, bacteria or viruses may directly invade the pharyngeal mucosa. This causes a local inflammatory reaction causing the symptoms of pharyngitis. In streptococcal pharyngitis, Strep pyogenes attaches to the mucosal epithelial and release extracellular toxins and proteases to prevent phagocytosis by the host macrophages. It also facilitates bacterial survival by producing a bacterial capsule.

Viral pharyngitis:

• Gradual onset
• Fever, malaise, sore throat/ throat pain
• Conjunctivitis, rhinitis, coryza, viral exanthem, diarrhoea

Streptococcal pharyngitis

• Headache, vomiting, abdominal pain, fever
• Tonsillar enlargement with exudates
• Cervical adenopathy
• Palatal petechiae and uvular oedema

Management

Investigations

Distinguishing between respiratory viral causes and Group A Streptococcus is important as management differs

• If symptoms strongly suggestive of viral URTI, diagnosis of viral pharyngitis can be made clinically

• If symptoms strongly suggestive of GAS pharyngitis, diagnosis should be made via microbiologic testing (rapid DNase antigen detection testing)

If unclear - use modified CENTOR criteria can be used

• Presence of tonsillar exudates (1)
• Tender/swollen anterior cervical adenopathy (1)
• Fever (1)

• Absence of cough (1)
• Age 3-14 (1), 15-44 (0), > 45 (-1)

Score: 0-1: Symptomatic treatment 2-3: Rapid antigen testing (If positive -> Tx with Abx) (If negative -> get throat culture) 4-5: Treatment with antibiotics Note: Positive throat culture warrants Abx therapy

Viral pharyngitis Symptomatic Treatment

• Reassure parents and carers
• Maintaining adequate hydration
• Warm saline gargles
• Eating a soft diet
• OTC products for symptomatic relief including antihistamines, decongestants, paracetamol

Streptococcal pharyngitis

• Supportive treatment to relieve fever and pain
• Antibiotic treatment (oral 20-50mg/kg/day penicillin for 10 days)
• Clindamycin or macrolides if allergic to penicillin

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Epiglottitis

Epiglottitis is the acute inflammation of the epiglottis and adjacent supraglottic structures. Epiglottitis can rapidly progress to life-threatening airway obstruction without prompt recognition and treatment. Haemophilus Influenza Type B (Hib) is historically the most common cause of epiglottitis. Since the development and introduction of the Hib vaccine, epiglottitis has become rare in children and adults. Nowadays, Group A Streptococcus is a more commonly responsible agent for this infection or the differential diagnosis of bacterial tracheitis, which can present with drooling and appearing toxic, secondary to a staphylococcal infection

Clinical Features

Pathophysiology

• High fever and lethargy (usually the first symptom)
• Sudden onset of inspiratory stridor and respiratory distress, tachypnea, anxiety, refusal to lie down
• Potential for rapid progression to complete airway obstruction

• Three Ds: Dysphagia, drooling, and distress
• Tripod position—hyperextended neck, leaning forward, mouth open
• Muffled voice (“hot potato” voice)
• Cough is typically absent
• Toxic appearing

Epiglottitis is due to a direct invasion of the epithelial layer by the pathogenic organism causing bacteraemia. Swelling of the epiglottis is due to oedema and accumulation of inflammatory cells in the potential space between the squamous epithelial layer and the epiglottal cartilage. As there is an abundant network of lymphatic and blood vessels, this facilitates the spread of infection and the inflammatory response. Supraglottic swelling reduces the size of the upper airway causing turbulent airflow during inspiration. This can rapidly progress to airway obstruction.

Investigations

Epiglottitis is a clinical diagnosis and laboratory, or other interventions should not delay timely control of the airway

Management

An epiglottitis team usually consists of a paediatrician, an anaesthetist and an ENT surgeon. If a child presents bacteraemic with drooling and signs consistent of an acute upper airway obstruction, it is imperative to secure the airways immediately. A rapid induction is performed followed by a direct laryngoscopy to determine if there is a swollen epiglottis. The swelling of the epiglottis can make it very difficult to see the vocal cords and it may not be possible to successfully insert an ETT. This is why the ENT surgeon needs to be standing ready to perform an emergency tracheostomy to ensure the patient's airway is maintained.

If patient is able to maintain airway

• Provide supplemental oxygen and maintain the child in a comfortable position
• Additional management after airway is assessed and managed
• IV ABx
• Cefotaxime: Children 150-200mg/kg/day IV given in divided doses every 6-8 hours OR
• Cetriaxone: Children with mild to moderate infections: 50-75mg/kg/day IV given in divided doses every 12-24 hours

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Pertussis (Whooping Cough)

Pertussis (whooping cough) is a highly infectious disease caused by Bordetella pertussis, a gram negative bacteria. The disease is mainly transmitted via airborne droplets. Patients can be infectious for several weeks if left untreated. Pertussis is typically a childhood disease, particularly in children <1 year of age. These children often present with a distinctive barking cough associated with an inspiratory whoop. Younger infants may not develop this typical cough and often present with apnoea and cyanosis instead.

Pathophysiology

Indications for Hospitalisation

Bordetella pertussis is highly contagious and transmitted via aerosolized respiratory droplets. After inhalation, the organism adheres to the ciliated respiratory epithelial cells of the upper respiratory tract and nasopharynx causing local tissue damage.

• Respiratory distress e.g. tachypnoea, retractions, nasal flaring, increased work of breathing
• Inability to breathe
• Pneumonia
• Cyanosis or apnoea

• Seizures

Clinical Features

• Suspect pertussis if child has paroxysmal cough with skin colour changes

Three identifiable stages – catarrhal, paroxysmal, convalescent1. Catarrhal (1-2 weeks)

• Coryzal symptoms (rhinorrhoea, lacrimation, dry cough) similar to a viral upper respiratory infection

2. Paroxysmal (2-8 weeks)

• Cough continues for many weeks to months, increasing in severity
• Child may gag, develop cyanosis, appear to be struggling to breathe
• Child appears well between cough paroxysms
• Cough may be more bothersome at night
• “Whoop” or noise made by forced inspiratory effort that follows the cough may not always be present
• Post tussive vomiting – moderately sensitive and specific for pertussis

3. Convalescent

• Cough subsides over several weeks to months

Management

Investigations

• A diagnosis can be made based on history and examination findings . However, if possible, laboratory tests should be performed to confirm the diagnosis.

• Leucocytosis and lymphocytosis. The most common finding is a lymphocytosis of > 11.0 x 10 /L

• Culture or PCR: Samples from deep nasopharyngeal aspiration or posterior nasopharyngeal swab

Macrolide antibiotics for patient and household contacts

• Infants < 1 months: Azithromycin

Supportive Care

• Fluids and nutrition
• Avoid triggers for paroxysmal cough including exercise, cold temperatures, nasopharyngeal suctioning
• Isolation until 5 days of therapy

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Foreign Body Aspiration

Foreign body aspiration occurs when a foreign body enters the airways. It should be suspected in children who have an acute onset of lower respiratory symptoms or those who do not respond to standard treatment of other suspected etiologies. The signs and symptoms will vary depending upon the site of impaction, degree of blockage and type of object. Children between one and three years of age are at the highest risk for FBA.

Pathophysiology

The narrowest portion of the paediatric airway is at the cricoid ring. If a foreign body obstructs the upper airway, it results in respiratory distress with severe retractions and stridor. If a foreign body obstructs the lower airway (80%), symptoms may be subtle

Clinical Features (Physical Exam)

Clinical Features (History)

• Patients or carers may not volunteer the history of possible inhalation as many foreign body aspirations are not witnessed
• Coughing or choking episodes while eating solid foods (e.g peanuts) or while sucking a small plastic toy or object
• A history of choking, even if it occurred days or weeks before presentation, can be highly suggestive of FBA

May be no physical signs, or asymmetrical chest movement, tracheal deviation, wheeze, reduced breath sounds over whole or part of one lung

Management

Investigations

Inspiratory and expiratory CXR (from nasopharynx to chest) Look for: An opaque foreign body, segmental or lobar collapse, localised emphysema in expiration CT is possible for: Asymptomatic patients or symptomatic but stable patients who have normal or inconclusive CXR but an ongoing clinical suspicion of FBA

Rigid bronchoscopy is indicated for all patients with suspected inhaled foreign body to identify and remove the objectParent and carer education

• Hard and/or round foods (e.g hard candy, grapes, raisins, popcorn) should not be offered to children < 4 years old
• All meals for young children should be supervised by an adult
• Children should be taught to chew their food well
• Avoid toys with small parts, keep small household items or coins out of reach

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Cystic Fibrosis

Cystic fibrosis is the most common severely life-shortening genetic disease in Caucasians. It is an autosomal recessive disorder with a carrier rate of 1 in 25 and an incidence of approximately 1 in 2500 live births.

Pathophysiology

• Autosomal recessive disorder in which there is a mutated "cystic fibrosis transmembrane conductance regulator" (CFTR) protein on Chromosome 7. Many different genotypes and phenotypes of cystic fibrosis have been identified over the last 20 years including df508, G552X, R117H etc.
• Normally, the CFTR is a channel protein that pumps chloride ions into various secretion. Those chloride ions draw water into the secretions, which helps to thin out the secretion.
• The most common mutation is ∆F508 (70%), causing the CFTR protein not to make it to the cell membrane.
• Without CFTR protein on the epithelial surface, chloride ions aren’t pumped into the secretions, leaving the secretions thick and sticky.
• This mutated CFTR protein causes secretions to be abnormally thick and sticky, principally involving the lungs and pancreas (as well as the liver, intestine and reproductive tract)

Clinical Features

• The most common presentation now is without symptoms and by newborn screening
• Typical presentation of poor weight gain, loose, foul-smelling, bulky stools; and a voracious appetite

Gastrointestinal/Nutritional

• Failure to thrive
• Foul smelling stools, bloating, abdominal pain
• Meconium Ileus (10%)
• Constipation, rectal prolapse
• Intestinal impaction and obstruction
• Pancreatitis insufficiency

Malabsorption with steatorrhea

• Fat-soluble vitamin deficiency (A, D, E, K)

• Glucose intolerance

Respiratory

• Cough (persistent and productive)
• Wheezing, dypsnoea, exercise tolerance
• Bronchiectasis, recurrent pneumonia
• Sinusitis, nasal polyps
• Reactive airway diseases, haemoptysis
• Nail clubbing

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Cystic Fibrosis

Investigations

• Newborn screening detects 90% of affected babies. All babies have a heel prick blood sample taken on Day 2-4 of life which measures the pancreatic enzyme trypsinogen. If the trypsinogen level is above the 99th percentile, it is analysed for the most common CF mutations.

• If not detected via the heel prick test, to diagnose CF, both of the following criteria must be met :

1. Clinical symptoms consistent with CF in at least one organ system, or positive newborn screen or having a sibling with CF AND2. Evidence of cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction (any of the following)

• Elevated sweat chloride ≥60 mmol/L
• Presence of two disease-causing mutations in the CFTR gene, one from each parental allele

• Abnormal nasal potential difference (NPD)

Management

• Currently no cure for CF, treatment aims to slow progression of the disease and can include:

Respiratory

• Chest physiotherapy
• Inhalations via a compressed air pump (aerosolised mucolytics) and nebuliser

Pancreatic/Digestive

• Enzyme replacement capsules with meals and snacks
• Pancreatic enzyme replacement therapy (containing lipase, amylase and protease)
• High calorie, high protein diet
• Percutaneous endoscopic gastrostomy (PEG) – a procedure in which a flexible feeding tube is placed through the abdominal wall and into the stomach
• Supplementary vitamins - A, D, E, K
• Salt supplements

• Ivacaftor (potentiator of CFTR protein) to target the abnormal the CFTR protein to restore appropriate enzymatic activity. More recently, a newer drug, TriKaftor, has now been proven effective for df508 deletion mutation.

• Antibiotics - azithromycin (has an anti-inflammatory as well as antibiotic effect)
• Regular exercise

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Bacterial Tracheitis

Bacterial tracheitis is an invasive exudative bacterial infection of the soft tissues of the trachea. In most cases, it occurs in previously health children during the first six years of life after a viral respiratory tract infection. The most common cause of bacterial tracheitis is staphylococcus aureus.

Pathophysiology

The larynx of a normal healthy individual is often colonised with bacteria. Some of these bacteria can potentially become pathogens (e.g. Staphylococcal aureus, Streptococcus pneumoniae, Pseudomonas aeruginosa) that can extend transiently into the trachea. Bacterial tracheitis generally occurs in the setting of prior airway mucosal damage, for example, a preceding viral infection. The bacterial infection of the soft tissues of the trachea leads to a rapidly progressive upper airway obstruction.

Clinical Features

• Prodromal symptoms & signs suggestive of a viral respiratory tract infection for 1-3 days before severe signs of disease (e.g. SOB and stridor, respiratory distress)
• Presentation very similar to croup, however: differentiation can be made by the presence of high fever, toxicity, inspiratory stridor (constant), purulent sputum

• Cough (membranous exudates may be expectorated)

• Respiratory distress
• Hoarseness or voice changes
• Odynophagia or dysphagia
• Drooling is uncommon but may be present

Management

Investigations

• A presumptive diagnosis can be made based on acute onset of airway obstruction with consistent clinical and radiographic features in the setting of a preceding viral upper respiratory infection
• Direct visualisation of the airway via laryngoscopy and tracheobronchoscopy is the definitive diagnosis of bacterial tracheitis

Assess the need for intubationSupportive respiratory care

• Supplemental oxygen
• Nebulised epinephrine if stridor is noted
• Fluid resuscitation (if needed)
• Antibiotic therapy

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Pneumonia

Pneumonia is a respiratory tract infection resulting in inflammation of lung parenchyma. It is more common in children < 5 years of age than in older children and adolescents. The pathogens responsible vary according to the age of the child and the setting in which the infection was acquired. (community vs hospital acquired)

Pathophysiology

Common Pathogens

Newborns

• Escherichia coli
• Group B streptococcus
• Streptococcus pneumoniae

• Haemophilus influenzae

Children (4 weeks - 18 years)

• C. trachomatis
• C. pneumoniae (young children & adolescents)
• S. pneumoniae

• Respiratory syncytial virus (RSV)
• Mycoplasma

Viruses: RSV, influenza, parainfluneza, adenovirus

Pneumonia usually follows an upper respiratory tract infection that allows the invasion of the lower respiratory tract by pathogens such as bacteria or viruses that trigger the immune response and produce inflammation. The lung parenchyma fill with WBC, cellular debris and fluid resulting in decreased lung compliance and increased resistance. Smaller airways become obstructed potentially resulting in collapse of distal air spaces. :

Clinical Features (Physical Exam)

Clinical Features (History)

• General appearance: For young infants - assess ability to feed, vocalise and to be consoled
• Fever
• Tachypnea
• Respiratory distress (e.g. increased work of breathing, use of accessory muscles, nasal flaring)
• Auscultation: Crackles, decreased breath sounds, dullness to percussion

Neonates & young Infants

• Difficulty feeding
• Restlessness
• Fussiness

Older children & adolescents

• Cough
• Fever
• Abdominal pain (due to referred pain from lower lobes)

Management

Investigations

The decision to admit must be individualised based on age, underlying medical history and severity of illness

• Supportive care including adequate antipyresis, analgesia, respiratory support and hydration
• Infants <3 - 6 months: Admit to hospital for management
• Ages 6 months – 5 years: Oral amoxicillin
• Ages >5 years: Bacterial pneumonia - oral amoxicillin 7 days.

If M. pneumoniae – azithromycin, erythromycin or levofloxacin

• Routine laboratory evaluation and CXR is not necessary for children with mild uncomplicated lower respiratory tract infection who will be treated in outpatients
• CXR should be obtained if: Severe disease, exclusion of alternate diagnoses, history of recurrent pneumonia, evaluation for complications

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MULTIPLE CHOICE QUESTIONS

1. A two-year old girl presents with a persistent day and night time cough. On examination, she has a productive cough and generalised wheeze. Her growth chart is shown below: Which one of the following is the most appropriate diagnostic test?A. Spirometry B. Bronchoscopy C. Chest X-ray D. Heel prick test E. Sweat chloride estimation

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MULTIPLE CHOICE QUESTIONS

2. A 17 month old child presents to ED with noisy breathing and a barking cough. He is febrile and looks toxic. On further questioning, his parents have not noticed any drooling. and he seems to be taking sips of water without any visible pain. On physical exam, his temperature is 39.50C, oxygen saturation is 97%, inspiratory and expiratory stridor are present with markedly increased work of breathing associated with poor air entry in the lung bases bilaterally. Direct laryngoscopy reveals a normal looking epiglottis. What is the next most appropriate step in management for this patient? A. Intubation and IV Antibiotics B. Inhaled epinephrine and oral steroids C. Oral antibiotics and discharge D. A burst of nebulised Ventolin therapy each 10 minutes E. Observation only 3. A five-year old girl is brought to ED with an acute onset of a fever of 390C and a sore throat. She is alert but flushed in the cheeks and appears anxious. She is drooling and has a quiet inspiratory stridor. What is your immediate management? A. Examine the throat B. Obtain IV access and the relevant blood tests C. Order a chest x-ray and lateral view of the neck D. Prepare to establish an airway E. Inhaled epinephrine and oral steroids

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MULTIPLE CHOICE QUESTIONS

4. You are an intern on your first night shift at ED. A mother brings in her two-year old daughter as she was awakened by a barking cough just after midnight. On further questioning, the mother states that her daughter has had a mild runny nose for the past three days, but when she woke up she appeared anxious and was struggling to breathe so she decided to present the hospital. On examination, you notice that she has nasal flaring, marked retractions of the chest wall and noisy breathing on inspiration. What is the most likely diagnosis? A. Asthma B. Epiglottitis C. Bronchiolitis D. Croup E. Foreign body 5. A 16 month child presents to her GP with unilateral nasal obstruction and foul-smelling discharge. The right side of her nose is sore to touch. The rest of her physical examination was unremarkable. What is the most likely diagnosis? A. Foreign body B. Deviated septum C. Nasal polyps D. Blocked nose E. Impetigo

6. A premature 29 week old boy is admitted to intensive care for appropriate care. Until day five, he does well with life but then develops a widened pulse pressure, subcostal retraction and an increased respiratory rate. He does not appear cyanotic or in need or additional oxygen. Upon investigations, a CXR shows increased pulmonary vascular markings. On auscultation, there is a continuous murmur. Which medication will most likely alleviate his symptoms? A. Furosemide B. Epinephrine C. Digoxin D. Indomethacin E. Morphine

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MULTIPLE CHOICE QUESTIONS

7. Parents are concerned about their two-month old girl with Down syndrome. They have noted that she turns blue around the mouth sometimes and she has not been eating as much as before. On examination, there is a systolic ejection murmur and a split S2. Her ECG shows Right ventricular hypertrophy . What is the most likely diagnosis? A. Patent Ductus Arteriosus B. Truncus Arteriosus C. Atrial Septal Defect D. Transposition of the great vessels E. Eisenmenger syndrome 8. A two-week old baby presents to ED with a four-day history of poor feeding. On physical examination, it is noted that there are absent femoral pulses and there is BP differential in the upper and lower limbs. What is the most likely diagnosis? A. Coarctation of Aorta B. Tetralogy of Fallot C. Atrial Septal Defect D. Pulmonary Stenosis E. Aortic Stenosis 9. A six-day old baby is brough for a one-week review post birth. He was well at birth but later developed cyanosis, has not been feeding properly. Parents are worried that their baby might not be breathing properly. Auscultation reveals a harsh systolic murmur and CXR reveals a 'boot' shaped heart. What is the most likely diagnosis? A. Pulmonary Stenosis B. Tetralogy of Fallot C. Transposition of the great vessels D. Eisenmenger syndrome E. Atrio-Septal Ventricular Defects 10. You are a fourth year medical student attending your paediatrics placement. At placement you see a cyanotic baby and decide to revise over congenital heart diseases and their managements. All of the following conditions can benefit from a Prostaglandin E1 infusion except? A. Coarctation of the aorta B. Patent Ductus Arteriosus C. Transposition of the great vessels D. Pulmonary Stenosis E. Tetralogy of Fallot

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MCQ - ANSWERS

1. Answer: E – Sweat chloride estimationIn this scenario, the two-year old girl growth percentiles suggests failure to thrive. In addition to this, she has a productive cough and generalised wheeze. The most common cause of this combination of symptoms and signs would be cystic fibrosis. A sweat chloride estimation is the most definitive diagnostic test for cystic fibrosis.

2. Answer: A - Intubation and IV ABx In this scenario, the 17 month old child has bacterial tracheitis which is severe and life-threatening sequela of croup. A differential that must be included is epiglottitis, however the lack of drooling and dysphagia are the features which make this a case of bacterial tracheitis. It is essential to secure the airway and treat using IV antibiotics 3. Answer: D – Prepare to establish an airway. In this scenario, the five-year old girl has epiglottitis which is a life-threatening condition that can progress to upper airway obstruction. The course of the illness is acute and there are no prodromal symptoms. There is a sudden onset of sore throat, high fever, and toxicity. In addition to this, drooling, dysphagia, a muffled voice, and a characteristic tripod position may be seen. The immediate management is to prepare to establish an airway.

4. Answer: D – Croup In this scenario, the two-year old girl has croup. The physical examination signs described are those involving the airway above the point at which the trachea enters the neck and leaves the thorax. Croup can be caused by parainfluenza, influenza, RSV and other viruses. The treatment is usually supportive, but epinephrine and corticosteroids can be given. 5. Answer: A – Foreign body. In this scenario, the 16 month old child presents with unilateral nasal obstruction and foul smelling discharge. Unilateral nasal discharge in the presence of obstruction suggests the need to examine for foreign bodies. Children often insert small objects into their noses. At first, only local irritation occurs. Later, the symptoms increase to include worsening pain as well as a purulent malodorous discharge.

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MCQ - ANSWERS

6. Answer: D - IndomethacinIn this scenario, the boy is assumed to have an acyanotic congenital heart disease as there is no increased oxygen requirements. A continuous murmur with wide pulse pressures and increased pulmonary vascular markings is consistent with a PDA. The most beneficial treatment is to close the PDA with indomethacin.

7. Answer: C - Atrial Septal Defect In this scenario, the two-month old girl with has an Atrial septal defect. This defect is common amongst children with Down syndrome. The mother's concerns and clinical findings of an ejection murmur and an ECG showing right ventricular hypertrophy are consistent with the diagnosis.

8. Answer: A - Pulmonary Stenosis. In this scenario, the two-week old baby has coarctation of the aorta with classic presentation of absent or weak femoral pulses and a BP difference between the upper and lower limbs. Due to the location of coarctation at the ascending aorta, there is a BP difference in limbs. Coarctation can also be detected in older children with a similar presentation and added chest pain, claudication of lower limb and cold feet. 9. Answer: B – Tetralogy of Fallot In this scenario, the one-week old boy is noted to have become worse after birth. His history, auscultation findings and investigations are most consistent with a Tetralogy of Fallot. Although, Pulmonary Stenosis and septal defects are components of Tetralogy of Fallot, they alone are not responsible for the symptoms in this child. The combination of the four factors results in Tetralogy of Fallot. 10. Answer: B - Patent Ductus Arteriosus This question is testing your understanding of the pathophysiology being a congenital heart disease and the necessary treatments needed for survival. Coarctation of the Aorta, transposition of the great vessels, Pulmonary Stenosis and Tetralogy of Fallot all require a Patent Ductus Arteriosus for survival. Prostaglandin E1 infusion ensures that the ductus arteriosus remains open allowing for a L->R shunting of blood.

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