Paediatrics Flashcards
Name the 3 fetal shunts
ductus arteriosus
foramen ovale
ductus venosus
Describe the changes at birth that close the 3 fetal shunts
first breath expands alveoli, lowers pulmonary vascular resistant, lower pressure in right atrium closes foramen ovale -> fossa ovalis
Blood oxygenation causes decrease in prostaglandins, closes ductus arteriosus -> ligamentum arteriosum
Clamping of umbilical cord, ductus venosus-> ligamentum venosum
What is the purpose of the ductus venosus?
shunt connects the umbilical vein to the inferior vena cava and allows blood to bypass the liver
what is the purpose of the foramen ovale ?
shunt connects the right atrium with the left atrium and allows blood to bypass the right ventricle and pulmonary circulation
What is the purpose of the ductus arteriosus?
shunt connects the pulmonary artery with the aorta and allows blood to bypass the pulmonary circulation
What are the typical features of an innocent murmur?
Soft
Short
Systolic
Symptomless
Situation dependent, particularly if the murmur gets quieter with standing or only appears when the child is unwell or feverish
What features of a murmur would prompt further investigation ?
Murmur louder than 2/6
Diastolic murmurs
Louder on standing
Other symptoms such as failure to thrive, feeding difficulty, cyanosis or shortness of breath
What are the key investigations to establish the cause of a murmur and rule out abnormalities in a child?
ECG
Chest Xray
Echocardiography
What are 3 differentials for a pan-systolic murmur ?
Mitral regurgitation
Tricuspid regurgitation
Ventricular septal defect
Where can the murmur be heard with a ventricular septal defect?
left lower sternal border
Name 3 differentials for an ejection-systolic murmur
Aortic stenosis
Pulmonary stenosis
Hypertrophic obstructive cardiomyopathy
What murmur does an atrial septal defect cause?
mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border
fixed split second heart sound
What murmur does patent ductus arteriosus cause?
continuous crescendo-decrescendo “machinery” murmur
What murmur does tetralogy of Fallot cause?
ejection systolic murmur loudest at the pulmonary area
Name 4 cyanotic heart diseases
Ventricular septal defect (VSD)
Atrial septal defect (ASD)
Patent ductus arteriosus (PDA)
Transposition of the great arteries
When does the ductus arteriosus usually stop functioning and close?
normally stops functioning within 1-3 days of birth, and closes completely within the first 2-3 weeks of life
Give 2 risk factors for patent ductus arteriosus
Rubella
Prematurity
Describe the pathophysiology of heart failure in patent ductus arteriosus
Left to Right shunt leading to pulmonary hypertension which causes right sided heart strain and hypertrophy
What are the symptoms of patent ductus arteriosus?
SOB
Difficulty feeding
Poor weight gain
LRTI
How is patent ductus arteriosus diagnosed?
Echo
How is patent ductus arteriosus managed?
monitoring with echo
Surgical or trans-catheter closure
Describe the pathophysiology of atrial septal defects
left to right shunt -> right sided strain -> right heart failure -> pulmonary hypertension
what are the 2 walls that form the atrial septum?
septum primum and septum secondum
What are the 3 types of atrial septal defect?
Ostium secondum
Patent foramen ovale
Ostium prinum
State 4 complications of atrial septal defects
Stroke in the context of venous thromboembolism (see below)
Atrial fibrillation or atrial flutter
Pulmonary hypertension and right sided heart failure
Eisenmenger syndrome
What symptoms may be present in a child with an atrial septal defect?
Shortness of breath
Difficulty feeding
Poor weight gain
Lower respiratory tract infections
What are the management options for an atrial septal defect?
watch and wait (small)
transvenous catheter closure or open heart surgery
anticoagulants (in adults)
Name 2 genetic conditions associated with ventricular septal defects
Down’s syndrome
Turner’s syndrome
What are the typical symptoms of ventricular septal defects?
Poor feeding
Dyspnoea
Tachypnoea
Failure to thrive
What are the examination findings of a ventricular septal defect?
pan-systolic murmur more prominently heard at the left lower sternal border in the third and fourth intercostal spaces. There may be a systolic thrill on palpation.
what are patients with a ventricular septal defect at increased risk of?
infective endocarditis
what is the management of a ventricular septal defect?
watch and wait
transvenous catheter closure or open heart surgery
What are the 3 underlying lesions that can result in Eisenmenger syndrome
Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
Describe the pathophysiology of Eisenmenger syndrome
defect in heart causes left to right, over time pulmonary hypertension develops when it exceeds systemic pressure the shunt reverses and is right to left, deoxygenated blood bypasses lungs and causes cyanosis
What examination findings are associated with pulmonary hypertension ?
Right ventricular heave: the right ventricle contracts forcefully against increased pressure in the lungs
Loud P2: loud second heart sound due to forceful shutting of the pulmonary valve
Raised JVP
Peripheral oedema
What findings in Eisenmenger syndrome are related to the right to left shunt and chronic hypoxia ?
Cyanosis
Clubbing
Dyspnoea
Plethoric complexion
What is the only definitive treatment once Eisenmenger syndrome has developed?
heart-lung transplant
What is the medical management of Eisenmenger syndrome?
Oxygen
Treatment of pulmonary hypertension e.g. sildenafil
Treatment of arrhythmias
Treatment of polycythaemia with venesection
Prevention and treatment of thrombosis with anticoagulation
Prevention of infective endocarditis using prophylactic antibiotics
What is coarctation of the aorta?
narrowing of the aortic arch
What genetic condition is associated with coarctation of the aorta?
Turners syndrome
How may coarctation of the aorta present?
weak femoral pulses
systolic murmur
differences in blood pressure in limbs
tachypnoea
poor feeding
grey and floppy baby
legs and left arm may be underdeveloped
What is the management of critical coarctation of the aorta?
Prostaglandin E (to keep ductus arteriosus open) whilst waiting for surgery
What are the presenting symptoms of aortic valve stenosis?
fatigue, shortness of breath, dizziness and fainting.
Symptoms usually worse on exertion
What are the key examination findings in aortic valve stenosis?
ejection systolic murmur
crescendo-decrescendo that radiates to the carotids
ejection click
palpable thrill
slow rising pulse and narrow pulse pressure
What is the gold standard investigation for aortic valve stenosis?
echocardiogram
What are the treatment options for aortic valve stenosis?
Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement
what are some complications of aortic valve stenosis?
Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death, often on exertion
what conditions are associated with congenital pulmonary valve stenosis?
Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome
What are some symptoms of severe pulmonary valve stenosis?
fatigue on exertion, shortness of breath, dizziness and fainting
What are some signs of pulmonary valve stenosis?
Ejection systolic murmur heard loudest at the pulmonary area (second intercostal space, left sternal border)
Palpable thrill in the pulmonary area
Right ventricular heave due to right ventricular hypertrophy
Raised JVP with giant a waves
what is the gold standard diagnostic investigation in pulmonary valve stenosis?
echocardiogram
What are the 4 defects present in tetralogy of fallot?
Ventricular septal defect (VSD)
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy
What are the risk factors for tetralogy of fallot?
Rubella infection
Increased age of the mother (over 40 years)
Alcohol consumption in pregnancy
Diabetic mother
What is the investigation of choice for diagnosing tetralogy of fallot?
echocardiogram
What is the characteristic CXR finding in tetralogy of fallot?
“boot shaped” heart due to right ventricular thickening
what are some signs and symptoms of tetralogy of fallot?
Cyanosis
Clubbing
Poor feeding
Poor weight gain
Ejection systolic murmur heard loudest in the pulmonary area
“Tet spells”
What are some precipitating factors for a tet spell?
waking, physical exertion or crying
what are the symptoms of a tet spell?
irritable, cyanotic and short of breath
What are the conservative measures for managing a tet spell?
Older children may squat when a tet spell occurs. Younger children can be positioned with their knees to their chest. Squatting increases the systemic vascular resistance. This encourages blood to enter the pulmonary vessels.
what are the medical options for managing a tet spell?
O2
bblockers
IV fluids
morphine
sodium bicarbonate
phenylephrine infusion
what are the management options for tetralogy of fallot?
neonates - prostaglandin infusion to maintain ductus arteriosus
definitive - open heart surgery
what is Ebstein’s anomaly?
congenital heart condition where the tricuspid valve is set lower in the right side of the heart (towards the apex), causing a bigger right atrium and a smaller right ventricle
what conditions are associated with ebstein’s anomaly
right to left shunt via atrial septal defect
Wolff-Parkinson-White syndrome
What are the presenting features of Ebstein’s Anomoly ?
Evidence of heart failure (e.g. oedema)
Gallop rhythm heard on auscultation characterised by the addition of the third and fourth heart sounds
Cyanosis
Shortness of breath and tachypnoea
Poor feeding
Collapse or cardiac arrest
How is ebstein’s anomoly diagnosed?
Echocardiogram
what is the management of ebstein’s anomoly?
treating arrhythmias and heart failure.
Prophylactic antibiotics
Surgical correction of defect
what is transposition of the great arteries?
condition where the attachments of the aorta and the pulmonary trunk to the heart are swapped
what defects are associated with transposition of the great arteries?
Ventricular septal defect
Coarctation of the aorta
Pulmonary stenosis
How does transposition of the great arteries present ?
usually picked up on antenatal scans
cyanosis
within a few weeks of life they will develop respiratory distress, tachycardia, poor feeding, poor weight gain and sweating
what is the management of transposition of the great arteries?
prostaglandin infusion to maintain ductus arteriosus
balloon septostomy
open heart surgery
what is bronchiolitis?
inflammation and infection in the bronchioles
what is the most common cause of bronchiolitis?
Respiratory syncytial virus
What are the presenting features of bronchiolitis?
Coryzal symptoms
signs of respiratory distress
dyspnoea
tachypnoea
poor feeding
mild fever
apnoeas
wheeze and crackles on auscultation
what are the signs of respiratory distress?
raised respiratory rate
use of accessory muscles (sternocleidomastoid, abdominal and intercostal)
Intercostal and subcostal recessions
nasal flaring
Head bobbing
tracheal tug
cyanosis
abnormal airway noises
children how have had bronchiolitis as infants are more likely to have what during childhood?
viral induced wheeze
When should you admit a child with bronchiolitis?
Aged under 3 months or any pre-existing condition such as prematurity, Downs syndrome or cystic fibrosis
50 – 75% or less of their normal intake of milk
Clinical dehydration
Respiratory rate above 70
Oxygen saturations below 92%
Moderate to severe respiratory distress, such as deep recessions or head bobbing
Apnoeas
Parents not confident in their ability to manage at home or difficulty accessing medical help from home
what is the management of bronchiolitis?
Ensuring adequate intake
Saline nasal drops and nasal suctioning
Supplementary oxygen if the oxygen saturations remain below 92%
Ventilatory support if required
what are the most helpful signs of poor ventilation on capillary blood gas?
Rising pCO2
falling pH
respiratory acidosis
what can be given as prevention against bronchiolitis?
Palivizumab
typical features of viral-induced wheeze (as opposed to asthma) are:
Presenting before 3 years of age
No atopic history
Only occurs during viral infections
how does viral-induced wheeze present ?
Presenting before 3 years of age
No atopic history
Only occurs during viral infections
how does an acute asthma exacerbation present?
Progressively worsening shortness of breath
Signs of respiratory distress
Fast respiratory rate
Expiratory wheeze on auscultation heard throughout the chest
The chest can sound “tight” on auscultation, with reduced air entry
what are the features of a moderate asthma attack?
peak flow >50% predicted
Normal speech
What are the features of a severe asthma attack?
peak flow <50% predicted
saturations <92%
Unable to complete sentences in one breath
signs of respiratory distress
resp rate >40 (1-5yrs), >30 (>5yrs)
heart rate >140 (1-5yrs), >125 (>5yrs)
What are the features of a life threatening asthma attack?
peak flow <33% predicted
Saturations <92%
Exhaustion and poor respiratory effort
hypotension
silent chest
cyanosis
altered consciousness/confusion
What is the stepwise treatment using bronchodilators in acute asthma and viral induced wheeze?
Inhaled or nebulised salbutamol (a beta-2 agonist)
Inhaled or nebulised ipratropium bromide (an anti-muscarinic)
IV magnesium sulphate
IV aminophylline
What is the stepwise management of acute asthma or viral induced wheeze?
Salbutamol inhalers via a spacer device: starting with 10 puffs every 2 hours
Nebulisers with salbutamol / ipratropium bromide
Oral prednisone (e.g. 1mg per kg of body weight once a day for 3 days)
IV hydrocortisone
IV magnesium sulphate
IV salbutamol
IV aminophylline
when can discharge be considered following an acute asthma exacerbation?
he child is well on 6 puffs 4 hourly of salbutamol
what presenting symptoms suggest a diagnosis of asthma?
Episodic symptoms with intermittent exacerbations
Diurnal variability, typically worse at night and early morning
Dry cough with wheeze and shortness of breath
Typical triggers
A history of other atopic conditions such as eczema, hayfever and food allergies
Family history of asthma or atopy
Bilateral widespread “polyphonic” wheeze heard by a healthcare professional
Symptoms improve with bronchodilators
what are some typical triggers of asthma?
Dust (house dust mites)
Animals
Cold air
Exercise
Smoke
Food allergens (e.g. peanuts, shellfish or eggs)
What investigations can be carried out to aid with the diagnosis of asthma?
Spirometry with reversibility testing (in children aged over 5 years)
Direct bronchial challenge test with histamine or methacholine
Fractional exhaled nitric oxide (FeNO)
Peak flow variability measured by keeping a diary of peak flow measurements several times a day for 2 to 4 weeks
What are the stages of medical therapy of chronic asthma in children under 5?
Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
Add a low dose corticosteroid inhaler or a leukotriene antagonist (i.e. oral montelukast)
Add the other option from step 2.
Refer to a specialist.
What is the medical therapy of chronic asthma in children 5-12yrs?
Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
Add a regular low dose corticosteroid inhaler
Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response.
Titrate up the corticosteroid inhaler to a medium dose. Consider adding:
Oral leukotriene receptor antagonist (e.g. montelukast)
Oral theophylline
Increase the dose of the inhaled corticosteroid to a high dose.
Referral to a specialist. They may require daily oral steroids.
Describe inhaler technique with a spacer
Assemble the spacer
Shake the inhaler (depending on the type)
Attach the inhaler to the correct end
Sit or stand up straight
Lift the chin slightly
Make a seal around the spacer mouthpiece or place the mask over the face
Spray the dose into the spacer
Take steady breaths in and out 5 times until the mist is fully inhaled
what investigations may be done in a child with recurrent lower respiratory tract infections?
FBC
CXR
serum immunoglobulins
Immunoglobulin G to previous vaccines
Sweat test
HIV test
what age group is typically affected by croup?
6 months to 2 years
What is the classic cause of croup that typically improves in less than 48hrs and responds well to dexamethasone?
Parainfluenza virus
name 4 causes of croup
Parainfluenza
Influenza
Adenovirus
Respiratory Syncytial Virus (RSV)
what is croup?
upper respiratory tract infection causing oedema in the larynx
what are the presenting features of croup?
Increased work of breathing
“Barking” cough, occurring in clusters of coughing episodes
Hoarse voice
Stridor
Low grade fever
What is the management of croup?
oral dexamethasone
Oxygen
Nebulised budesonide
Nebulised adrenalin
Intubation and ventilation
what is the typical cause of epiglottitis?
haemophilus influenza type B
what are the presenting features of epiglottitis?
Patient presenting with a sore throat and stridor
Drooling
Tripod position, sat forward with a hand on each knee
High fever
Difficulty or painful swallowing
Muffled voice
Scared and quiet child
Septic and unwell appearance
what would a lateral x-ray of the neck show in epiglottitis?
thumb sign
what is the management of epiglottitis?
do not distress patient
alert senior paediatrician and anaesthetist
ensure airway is secure
IV antibiotics e.g. ceftriaxone
Steroids e.g. dexamethasone
what is laryngomalacia?
part of the larynx above the vocal cords (the supraglottic larynx) is structured in a way that allows it to cause partial airway obstruction
what is the key presenting feature of laryngomalacia?
inspiratory stridor
what is the cause of whooping cough ?
Bordetella pertussis (a gram negative bacteria)
what are the presenting features of whooping cough?
mild coryzal symptoms
severe coughing fits - paroxysmal
inspiratory whoop
How is whooping cough diagnosed?
nasopharyngeal or nasal swab with PCR testing or bacterial culture can confirm the diagnosis within 2 to 3 weeks of the onset of symptoms.
Where the cough has been present for more than 2 weeks patients can be tested for the anti-pertussis toxin immunoglobulin G
what is the management of whooping cough?
notify public health
supportive care
Macrolide antibiotics e.g. azithromycin
prophylactic antibiotics to close contacts in vulnerable groups
how long do the symptoms of whooping cough typically last for?
8 weeks
what is a key complication of whooping cough?
bronchiectasis
what are the features of chronic lung disease of prematurity?
Low oxygen saturations
Increased work of breathing
Poor feeding and weight gain
Crackles and wheezes on chest auscultation
Increased susceptibility to infection
what can be done to prevent chronic lung disease of prematurity?
Corticosteroids e.g. betamethasone to mothers showing signs of premature labour
CPAP rather than intubation
Caffine
Not over-oxygenating
what is the management of chronic lung disease of prematurity ?
sleep study to assess oxygen
oxygen saturations
O2 therapy
monthly palivizumab
how is a diagnosis of chronic lung disease of prematurity made?
chest xray changes and when the infant requires oxygen therapy after they reach 36 weeks gestational age.
what is the inheritance pattern of cystic fibrosis?
autosomal recessive
a genetic mutation on which mutation causes cystic fibrosis
7
what proportion of the population are carriers and what proportion have cystic fibrosis?
carriers = 1/25
have CF= 1/2500
what are the key consequences of the cystic fibrosis mutation?
thick pancreatic and biliary secretions
low volume thick airway secretions
Congenital bilateral absence of the vas deferens
how is CF screened for at birth?
newborn bloodspot test
what is often the first sign of cystic fibrosis?
Meconium ileus
What are the symptoms of cystic fibrosis?
Chronic cough
Thick sputum production
Recurrent respiratory tract infections
Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes
Abdominal pain and bloating
Parents may report the child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat
Poor weight and height gain (failure to thrive)
what are some signs of cystic fibrosis?
Low weight or height on growth charts
Nasal polyps
Finger clubbing
Crackles and wheezes on auscultation
Abdominal distention
what are some causes of clubbing in children?
Hereditary clubbing
Cyanotic heart disease
Infective endocarditis
Cystic fibrosis
Tuberculosis
Inflammatory bowel disease
Liver cirrhosis
What are the 3 key methods for diagnosing cystic fibrosis?
Newborn blood spot testing
sweat test = gold standard
Genetic testing for CFTR gene
Name 4 common colonisers in people with cystic fibrosis
Staphylococcus aureus
Haemophilus influenza
Klebsiella pneumoniae
Escherichia coli
Burkhodheria cepacia
Pseudomonas aeruginosa
How is pseudomonas colonisation treated in patients with cystic fibrosis?
long term nebulised antibiotics such as tobramycin. Oral ciprofloxacin is also used.
What are the aspects of management in cystic fibrosis?
Chest physiotherapy
Exercise
High calorie diet
CREON tablets
prophylactic flucloxacillin
treat chest infections
bronchodilators
Nebulised DNase
Nebulised hypertonic saline
vaccinations enc. pneumococcal and varicella
What conditions do people with cystic fibrosis need monitoring and screening for?
diabetes, osteoporosis, vitamin D deficiency and liver failure
What is the inheritance pattern of primary ciliary dyskinesia?
autosomal recessive condition
what is Kartagner’s triad in primary ciliary dyskinesia?
Paranasal sinusitis
Bronchiectasis
Situs Inversus
what is primary ciliary dyskinesia ?
ysfunction of the motile cilia around the body, most notably in the respiratory tract. This leads to a buildup of mucus in the lungs, providing a great site for infection that is not easily cleared
also affects the cilia in the fallopian tubes of women and the tails (flagella) of the sperm in men
what is situs inversus?
all the internal (visceral) organs are mirrored inside the body
What is a key risk factor for primary ciliary dyskinesia?
consanguinity in the parents
what are some medical causes of abdominal pain in children?
Constipation
Urinary tract infection
Coeliac disease
Inflammatory bowel disease
Irritable bowel syndrome
Mesenteric adenitis
Abdominal migraine
Pyelonephritis
Henoch-Schonlein purpura
Tonsilitis
Diabetic ketoacidosis
Infantile colic
What are some surgical causes of abdominal pain in children?
Appendicitis
Intussusception
Bowel obstruction
Testicular torsion
What are some red flags for serious abdominal pain in children?
Persistent or bilious vomiting
Severe chronic diarrhoea
Fever
Rectal bleeding
Weight loss or faltering growth
Dysphagia (difficulty swallowing)
Nighttime pain
Abdominal tenderness
What are the features of an abdominal migraine?
central abdominal pain lasting more than 1 hour
There may be associated:
Nausea and vomiting
Anorexia
Pallor
Headache
Photophobia
Aura
what is the main preventative medication for abdominal migraines?
Pizotifen
What are some typical features in a history and examination that suggest constipation?
Less than 3 stools a week
Hard stools that are difficult to pass
Rabbit dropping stools
Straining and painful passages of stools
Abdominal pain
Holding an abnormal posture, referred to as retentive posturing
Rectal bleeding associated with hard stools
Faecal impaction causing overflow soiling, with incontinence of particularly loose smelly stools
Hard stools may be palpable in abdomen
Loss of the sensation of the need to open the bowels
what is Encopresis?
faecal incontinence
at what age is faecal incontinence considered pathological?
4 years
what are some causes of faecal incontinence?
Chronic constipation
Spina bifida
Hirschprung’s disease
Cerebral palsy
Learning disability
Psychosocial stress
Abuse
What lifestyle factors can contribute to constipation?
Habitually not opening the bowels
Low fibre diet
Poor fluid intake and dehydration
Sedentary lifestyle
Psychosocial problems
What are some red flags for constipation in children?
Not passing meconium within 48 hours of birth
Neurological signs or symptoms
Vomiting
Ribbon stool
Abnormal anus
Abnormal lower back or buttocks
Failure to thrive
Acute severe abdominal pain and bloating
What are some complications of constipation?
Pain
Reduced sensation
Anal fissures
Haemorrhoids
Overflow and soiling
Psychosocial morbidity
what are the management options for constipation ?
Correct any contributing factors
Movicol
disimpaction regime if required
encourage and praise visiting the toilet
what causes GORD in babies?
immaturity of the lower oesophageal sphincter
by what age do 90% of infants stop having reflux by?
1 year
What are some signs of problematic reflux?
Chronic cough
Hoarse cry
Distress, crying or unsettled after feeding
Reluctance to feed
Pneumonia
Poor weight gain
What advise can be given to help reflux?
Small, frequent meals
Burping regularly to help milk settle
Not over-feeding
Keep the baby upright after feeding (i.e. not lying flat)
what are the management options for problematic reflux?
Gaviscon mixed with feeds
Thickened milk or formula (specific anti-reflux formulas are available)
Proton pump inhibitors (e.g., omeprazole) where other methods are inadequate
what is Sandifer’s syndrome?
rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants
what is the key symptom of pyloric stenosis?
projectile vomiting
what may be felt on examination in pyloric stenosis?
olive mass in upper abdomen
What will blood gas analysis show in pyloric stenosis?
hypochloric (low chloride) metabolic alkalosis
How is a diagnosis of pyloric stenosis made?
abdominal ultrasound to visualise the thickened pylorus
what is the treatment of laparoscopic pyloromyotomy
laparoscopic pyloromyotomy
What is the main concern in gastroenteritis?
Dehydration
what are 2 common causes of viral gastroenteritis?
Rotavirus
Norovirus
What are the symptoms of e.coli gastroenteritis?
abdominal cramps, bloody diarrhoea and vomiting
What is a common cause of travellers diarrhoea?
Campylobacter Jejuni
What is biliary atresia?
congenital condition where a section of the bile duct is either narrowed or absent
How does biliary atresia present?
persistent jaundice, lasting more than 14 days in term babies and 21 days in premature babies
what is the initial investigation for biliary atresia and what will it show?
conjugated and unconjugated bilirubin.
A high proportion of conjugated bilirubin
what is the management of biliary atresia?
Kasai portoenterostomy
what are some causes of intestinal obstruction
Meconium ileus
Hirschsprung’s disease
Oesophageal atresia
Duodenal atresia
Intussusception
Imperforate anus
Malrotation of the intestines with a volvulus
Strangulated hernia
How does bowel obstruction present?
Persistent vomiting. This may be bilious
Abdominal pain and distention
Failure to pass stools or wind
Abnormal bowel sounds.
What is the initial investigation for bowel obstruction?
abdominal xray
may show dilated loops of bowel proximal to the obstruction and absence of air in the rectum
what is the management of bowel obstruction?
nil by mouth
NG tube
IV fluids
Treat underlying cause
what is Hirschsprung’s disease?
congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum.
what syndromes are associated with Hirschsprung’s disease?
Downs syndrome
Neurofibromatosis
Waardenburg syndrome
Multiple endocrine neoplasia type II
what are the presenting features of Hirschsprungs disease?
Delay in passing meconium (more than 24 hours)
Chronic constipation since birth
Abdominal pain and distention
Vomiting
Poor weight gain and failure to thrive
What is Hirschsprung-Associated Enterocolitis
inflammation and obstruction of the intestine typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea
can lead to toxic megacolon and perforation of the bowel. It requires urgent antibiotics, fluid resuscitation and decompression
what investigations can be done to diagnose Hirshsprungs disease ?
Abdominal xray
Rectal biopsy
what will rectal biopsy show in Hirschsprungs disease?
absence of ganglionic cells
what is the definitive management of Hirschsprungs disease?
surgical removal of the aganglionic section of bowel
What conditions are associated with intussusception?
Concurrent viral illness
Henoch-Schonlein purpura
Cystic fibrosis
Intestinal polyps
Meckel diverticulum
What are the presenting symptoms of intussusception?
Severe, colicky abdominal pain
Pale, lethargic and unwell child
“Redcurrant jelly stool”
Right upper quadrant mass on palpation. This is described as “sausage-shaped”
Vomiting
Intestinal obstruction
how is intussusception diagnosed ?
ultrasound or contrast enema
what is the management of intussusception?
therapeutic enemas
surgical reduction
what are some complications of intussusception?
Obstruction
Gangrenous bowel
Perforation
Death
when is the peak age of appendicitis?
10 to 20 years
where is McBurney’s point?
one third the distance from the anterior superior iliac spine (ASIS) to the umbilicus
what are the classic features of appendicitis?
pain (central -> RIF)
anorexia
N + V
Rovsing’s sign
guarding
rebound and percussion tenderness
what is Rovsing’s sign?
palpation of the left iliac fossa causes pain in the RIF
what does rebound and percussion tenderness suggest in appendicitis?
peritonitis caused by a ruptured appendix
what are some key differentials of appendicitis?
ectopic pregnancy
ovarian cysts
Meckel’s diverticulum
Mesenteric adenitis
appendix mass
how is appendicitis managed?
appendicectomy
what are some complications of an appendicectomy?
Bleeding, infection, pain and scars
Damage to bowel, bladder or other organs
Removal of a normal appendix
Anaesthetic risks
Venous thromboembolism
What are the principles of DKA management in children?
Correct dehydration evenly over 48 hours. This will correct the dehydration and dilute the hyperglycaemia and the ketones. Correcting it faster increases the risk of cerebral oedema.
Give a fixed rate insulin infusion. This allows cells to start using glucose again. This in turn switches off the production of ketones.
what are some features of adrenal insufficiency in babies?
Lethargy
Vomiting
Poor feeding
Hypoglycaemia
Jaundice
Failure to thrive
what are some features of adrenal insufficiency in older children?
Nausea and vomiting
Poor weight gain or weight loss
Reduced appetite (anorexia)
Abdominal pain
Muscle weakness or cramps
Developmental delay or poor academic performance
Bronze hyperpigmentation to skin in Addison’s
How do females with congenital adrenal hyperplasia present?
virilised genitalia
an enlarged clitoris due to the high testosterone levels
Patients with more severe congenital adrenal hyperplasia can present shortly after birth with what symptoms?
hyponatraemia, hyperkalaemia and hypoglycaemia.
This leads to signs and symptoms:
Poor feeding
Vomiting
Dehydration
Arrhythmias
what are some features of mild congenital adrenal hyperplasia in males?
Tall for their age
Deep voice
Large penis
Small testicles
Early puberty
what is the management of congenital adrenal hyperplasia ?
Cortisol replacement, usually with hydrocortisone, similar to treatment for adrenal insufficiency
Aldosterone replacement, usually with fludrocortisone
Female patients with “virilised” genitals may require corrective surgery
how may growth hormone deficiency present at birth or in neonates?
Micropenis (in males)
Hypoglycaemia
Severe jaundice
how may growth hormone deficiency present in older infants or children?
Poor growth, usually stopping or severely slowing from age 2-3
Short stature
Slow development of movement and strength
Delayed puberty
what is the main investigation in growth hormone deficiency?
growth hormone stimulation test
What is the management growth hormone deficiency?
Daily subcutaneous injections of growth hormone (somatropin)
Treatment of other associated hormone deficiencies
Close monitoring of height and development
what are the presenting features of congenital hypothyroidism ?
Prolonged neonatal jaundice
Poor feeding
Constipation
Increased sleeping
Reduced activity
Slow growth and development
what are some symptoms of a UTI in babies?
Fever
Lethargy
Irritability
Vomiting
Poor feeding
Urinary frequency
what is the management of a UTI in children?
<3m with fever = immediate IV antibiotics
>3m and systemically well = oral antibiotics
when should children with UTIs have ultrasound scans?
All children under 6 months with their first UTI should have an abdominal ultrasound within 6 weeks, or during the illness if there are recurrent UTIs or atypical bacteria
Children with recurrent UTIs should have an abdominal ultrasound within 6 weeks
Children with atypical UTIs should have an abdominal ultrasound during the illness
when should a DMSA scan be done?
4 – 6 months after the illness to assess for damage from recurrent or atypical UTIs
How is Vesico-ureteric reflux diagnosed?
micturating cystourethrogram (MCUG)
what is the management of vesico-ureteric reflux?
Avoid constipation
Avoid an excessively full bladder
Prophylactic antibiotics
Surgical input from paediatric urology
What can exacerbate vulvovaginitis?
Wet nappies
Use of chemicals or soaps in cleaning the area
Tight clothing that traps moisture or sweat in the area
Poor toilet hygiene
Constipation
Threadworms
Pressure on the area, for example horse riding
Heavily chlorinated pools
between what ages in vulvovaginitis common?
3 and 10 years
what are the presenting symptoms of vulvovaginitis?
Soreness
Itching
Erythema around the labia
Vaginal discharge
Dysuria (burning or stinging on urination)
Constipation
what is the management of vulvovaginitis?
Avoid washing with soap and chemicals
Avoid perfumed or antiseptic products
Good toilet hygiene, wipe from front to back
Keeping the area dry
Emollients, such as sudacrem can sooth the area
Loose cotton clothing
Treating constipation and worms where applicable
Avoiding activities that exacerbate the problem
What is the classic triad of Nephrotic syndrome?
Low serum albumin
High urine protein content (>3+ protein on urine dipstick)
Oedema
Apart from the classic triad what are 3 other feature that occur in patients with nephrotic syndrome?
Deranged lipid profile, with high levels of cholesterol, triglycerides and low density lipoproteins
High blood pressure
Hyper-coagulability, with an increased tendency to form blood clots
What is the most common cause of nephrotic syndrome in children?
minimal change disease
nephrotic disease can be secondary to what?
intrinsic kidney disease (focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis)
Systemic illness (HSP, diabetes, infection)
what is the management of minimal change disease?
corticosteroids
what is the general management of nephrotic syndrome?
High dose steroids (i.e. prednisolone)
Low salt diet
Diuretics may be used to treat oedema
Albumin infusions may be required in severe hypoalbuminaemia
Antibiotic prophylaxis may be given in severe cases
what may be used to manage nephrotic syndrome in steroid resistant children?
ACE inhibitors and immunosuppressants
What are some complications of nephrotic syndrome?
Hypovolaemia
Thrombosis
Infection
Acute or chronic renal failure
Relapse
what are the features of nephritis?
Reduction in kidney function
Haematuria: invisible or visible amounts of blood in the urine
Proteinuria: although less than in nephrotic syndrome
What are the 2 most common causes of Nephritis in children?
post-streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease).
How long after streptococcus infection does post-streptococcal glomerulonephritis occur?
1 – 3 weeks
what condition is related to IgA Nephropathy?
Henoch-Schonlein Purpura
what is the management of nephritis ?
supportive
may require immunosupression
when do most children get control of daytime and night time urination?
Most children get control of daytime urination by 2 years and night time urination by 3 – 4 years.
what is primary nocturnal enuresis?
where the child has never managed to be consistently dry at night
What are causes of primary nocturnal enuresis?
variation on normal development
overactive bladder
fluid intake
failure to wake
psychological distress
secondary causes e.g. chronic constipation
what is the management of primary nocturnal enuresis?
Reassurance
Lifestyle changes e.g. pass urine before bed
Positive reinforcement
Treat underlying causes
Enuresis alarms
Pharmacological treatment
What is secondary nocturnal enuresis?
where a child begins wetting the bed when they have previously been dry for at least 6 months
What are some causes of secondary nocturnal enuresis?
Urinary tract infection
Constipation
Type 1 diabetes
New psychosocial problems (e.g. stress in family or school life)
Maltreatment
What medications are there for enuresis?
Desmopressin
Oxybutynin
Imipramine
what is multicystic dysplastic kidney?
one of the baby’s kidneys is made up of many cysts while the other kidney is normal
what is a Wilms tumour ?
specific type of tumour affecting the kidney in children, typically under the age of 5 years
what are the presenting features of a Wilms tumour?
mass in abdomen
Abdominal pain
Haematuria
Lethargy
Fever
Hypertension
Weight loss
what is the initial investigation of a wilms tumour
ultrasound
what is the management of a Wilms tumour?
nephrectomy
adjuvant chemo/radiotherapy
what is a posterior urethral valve?
where there is tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output. It occurs in newborn boys.
what are the presenting features of a posterior urethral valve?
Difficulty urinating
Weak urinary stream
Chronic urinary retention
Palpable bladder
Recurrent urinary tract infections
Impaired kidney function
what investigations may be done for a posterior urethral valve?
Abdo ultrasound
MCUG
Cystoscopy
what is the management of a posterior urethral valve?
monitoring
temporary catheter
ablation
what percentage of boys have undescended testes at birth ?
5%
what are the risks of undescended testes in an older child?
testicular torsion
infertility
testicular cancer
what are the risk factors for undescended testes?
Family history of undescended testes
Low birth weight
Small for gestational age
Prematurity
Maternal smoking during pregnancy
what is the management of undescended testes?
watch and wait until 6m
seen by paeds urologist
Orchidopexy between 6 and 12 months
what is Hypospadias?
urethral meatus (the opening of the urethra) is abnormally displaced to the ventral side (underside) of the penis, towards the scrotum
what are some complications of hypospadias?
Difficulty directing urination
Cosmetic and psychological concerns
Sexual dysfunction
what is a hydrocele?
collection of fluid within the tunica vaginalis that surrounds the testes
what is a communicating hydrocele?
tunica vaginalis around the testicle is connected with the peritoneal cavity via a pathway called the processus vaginalis. This allows fluid to travel from the peritoneal cavity into the hydrocele, allowing the hydrocele to fluctuate in size
what are the examination findings of a hydrocele?
soft, smooth, non-tender swelling around one of the testes. The swelling will be in front of and below
transilluminate with light
what is the management of a hydrocele?
Ultrasound
if simple usually resolve within 2yrs
communicating require surgery
what cells produce surfactant?
type II alveolar cells
at what gestation do type II alveolar cells become mature enough to start producing surfactant?
24 and 34 weeks
what are the principles of neonatal resuscitation?
Warm the baby
calculate APGAR score (1,5 & 10 mins)
Stimulate breathing, head in neutral position, check for airway obstruction
Inflation breaths (2 cycles of 5 breaths, if no response then 30seconds of ventilation breaths) still no response start compressions
Chest compressions 3:1 ratio
prolonged hypoxia increases the risk of what in neonates?
hypoxic-ischaemic encephalopathy (HIE)
what are the features of the APGAR score ?
Appearance (blue centrally, blue extremities, pink)
Pulse (absent, <100, >100)
Grimmace (no, little, good response)
Activity (floppy, flexed, active)
Respiration (absent, slow/irregular, strong/crying)
what is the APGAR score measured out of?
10
what are the main aspects of normal care of the neonate after birth?
Skin to skin
Clamp the umbilical cord
Dry the baby
Keep the baby warm with a hat and blankets
Vitamin K
Label the baby
Measure the weight and length
when is the blood spot screening carried out?
day 5
what 9 conditions are tested for in blood spot screening?
Sickle cell disease
Cystic fibrosis
Congenital hypothyroidism
Phenylketonuria
Medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
Maple syrup urine disease (MSUD)
Isovaleric acidaemia (IVA)
Glutaric aciduria type 1 (GA1)
Homocystin
when is the newborn examination carried out?
72 hours after birth
repeated at 6-8 weeks by GP
how do you measure pre-ductal saturations?
baby’s right hand
how do you measure post-ductal saturations?
in either foot
what are some features of general appearance in a NIPE?
Colour (pink is good)
Tone
Cry
skin changes
What should you inspect from a babies head in a NIPE?
General appearance: size, shape, dysmorphology, caput succedaneum, cephalohaematoma and any facial injury
Head circumference
Anterior and posterior fontanelles
Sutures
Ears
Eyes: slight squints are normal, epicanthic folds can indicate Down’s, purulent discharge could indicate infection
Red reflex
Mouth: cleft lip or tongue tie
Put your little finger in their mouth to check the suckling reflect and feel the palate all the way back, checking for a cleft palate.
What should you inspect on the shoulder and arms in a NIPE?
Shoulder symmetry: check for a clavicle fracture
Arm movements: check for an Erbs palsy
Brachial pulses
Radial pulses
Palmar creases: a single palmar crease is associated with Down’s, but can be normal
Digits: check the number of digits and if the fingers are straight or curved (clinodactyly)
Use a sats probe on the right wrist for a pre-ductal reading
What should you inspect from the chest in a NIPE?
Oxygen saturations in the right wrist and a feet: 95% and above is normal
Observe breathing: look for respiration distress, symmetry and listen for stridor
Heart sounds: listen for murmurs, heart sounds, heart rate and identify which side the heart is on heart
Breath sounds: listen for symmetry, good air entry and added sounds
What should you inspect from the abdomen in a NIPE?
Observe the shape: a concave abdomen may indicate diaphragmatic hernia with abdominal contents in the chest
Umbilical stump: look for discharge, infection and a periumbilical hernia
Palpate for organomegaly, hernias or masses
What should you examine from the genitals in a NIPE?
Observe for the sex, ambiguity and any obvious abnormalities
Palpate testes and scrotum: check both are present and descended, check for hernias or hydroceles
Inspect the penis for hypospadias, epispadias and urination
Inspect the anus to check if it is patent
Ask about meconium and whether the baby has opened the bowel
What should you inspect from the legs in a NIPE?
Observe the legs and hips for equal movements, skin creases, tone and talipes
Barlows and Ortolani manoeuvres: check for clunking, clicking and dislocation of the hips
Count the toes
what should you inspect from the back in a NIPE?
Inspect and palpate the spine: look for curvature, spina bifida and a pilonidal sinus
What reflexes should be tested in a NIPE?
Moro reflex: when rapidly tipped backwards the arms and legs will extend
Suckling reflex: placing a finger in the mouth will prompt them to suck
Rooting reflex: tickling the cheek will cause them to turn towards the stimulus
Grasp reflex: placing a finger in the palm will cause them to grasp
Stepping reflex: when held upright and the feet touch a surface they will make a stepping motion
What is Caput Succedaneum ?
fluid (oedema) collecting on the scalp, outside the periosteum (able to cross suture lines)
What is a cephalohaematoma?
collection of blood between the skull and the periosteum (does not cross suture lines)
