Paediatrics: NOTES Flashcards

Question

What is stridor?

Answer 1

Noisy breathing that occurs due to obstructed air flow through a narrowed airway

Answer 2

Do not examine the throat (due to risk of closure). Assess degree of stridor and subcostal recession, RR, HR, LOC, pulse oximetry

Answer 3

Oral dexamethasone (0.15mg/kg). Croup responds well to steroids (can also use oral prednisolone or nebulized steroids)

Answer 4

Nebulised adrenaline with oxygen by facemask

Answer 5

Haemophilus influenza type B

Answer 6

URTI causing oedema in the larynx

Answer 7

unvaccinated child presenting with a fever, sore throat, difficulty swallowing, sitting forward (tripod position) and drooling. Scared and unwell. Muffled voice (can be called hot potato voice). Minimal cough

Answer 8

Epiglottitis

Answer 9

Intense swelling of the epiglottitis and surrounding tissues, associated with septicaemia. High risk of obstruction

Answer 10

Direct visualisation by airway trained staff, or xray. DO NOT EXAMINE THROAT DUE TO OBSTRUCTION RISK, DO NOT DISTRESS PATIENT

Answer 11

Lateral: thumb sign (swelling of the epiglottis) Anterior-posterior: steeple sign (subglottic narrowing)

Answer 12

Urgent airway management (intubation/tracheostomy). Blood culture. ABx

Answer 13

IV 2nd/3rd generation cephalosporin (cefuroxime, ceftriaxone, cefotaxime)

Answer 14

Rifampicin

Answer 15

Epiglottic abscess

Answer 16

Chronic inflammatory airway disease leading to variable airway obstruction

Answer 17

FHx, dust, animals, cold air, exercise, smoke, food allergens

Answer 18

atopy; eczema, hayfever, food allergies

Answer 19

Diurnal variability, dry cough, progressively worsening SOB, signs of respiratory distress, tachypnoea, expiratory wheeze, improvements of symptoms with bronchodilators

Answer 20

Bilateral widespread polyphonic wheeze (believed to represent many airways of different sizes vibrating from abnormal narrowing)

Answer 21

Silent chest; the airways are so tight it isn't possible for a child to move air through the airways to create a wheeze

Answer 22

Focal lesion, inhaled foreign body, infection

Answer 23

Moderate: >50% Severe: <50% Life-threatening: <33%

Answer 24

<50% peak flow, <92% oxygen saturations, unable to complete sentences in one breath, signs of respiratory distress, tachypnoea, tachycardia

Answer 25

<33% peak flow, <92% O2 sats, exhaustion and poor respiratory effort, hypotension, silent chest, cyanosis, altered consciousness/confusion

Answer 26

Over 5 years (requires a degree of cooperation from the child)

Answer 27

Hyperinflation, flattened hemi-diaphragms, peribronchial cuffing, atelectasis (partial or incomplete inflation of lung)

Answer 28

Hypokalaemia; manage serum K+. Can cause tachycardia and a tremor

Answer 29

Vial episodic wheezing (only in response to viral infections), multiple trigger wheeze (in response to multiple triggers, can develop into asthma over time), asthma

Answer 30

Thought to result from small airways being more likely to narrow and obstruct due to inflammation and immune responses to viral infection. Episodic, as often triggered by common cold viruses

Answer 31

Bronchial inflammation (oedema, execessive mucus production, infiltration with cells), bronchial hyperresponsiveness (exaggerated twitchiness to inhaled stimuli), airway narrowing (reversible airflow obstruction)

Answer 32

Salmeterol, formoterol

Answer 33

Inhaled corticosteroid (preventers; decrease airway inflammation, result in decreased symptoms)

Answer 34

Budesonide, beclametasone, fluticasone

Answer 35

Imapired growth, adrenal suppression, altered bone metabolism. Ensure to always monitor the child's growth

Answer 36

An anticholinergic dilator. Can be given to young infants when other bronchodilators ineffective, or for the tx of severe acute asthma

Answer 37

Montelukast

Answer 38

1. Salbutamol inhalers via spacer device 2. Nebulisers with salbutamol/ipratroium bromide 3. Oral prednisolone 4. IV hydrocortisone 5. IV magnesium sulfate 6. IV salbutamol 7. IV aminophylline

Answer 39

SABA: salbutamol

Answer 40

- less than 5 years: add LTRA (montelukast) - if more than 5 years, add a LABA. If poor response, can either increase ICS. If very poor response, stop LABA, add LTRA

Answer 41

Theophylline

Answer 42

After all other avenues explored, such as high dose inhaled corticosteroids, LTRA, theophylline

Answer 43

if after high dose inhaled bronchodilator therapy; - havent response: eg, still breathless or have tachypnoea - exhausted - still reducing peak flow - <92% O2 sat

Answer 44

Moderate: some intercostal recession Severe: accessory neck muscles Life threatening: poor respiratory effor

Answer 45

RSV or rhinovirus

Answer 46

Poiseuille's law; small diameter of the airwar, proportionally larger restriction in airflow

Answer 47

- asthma is a clinical diagnosis, and rarely made before the age of 5. Viral induced wheeze is a symptom rather than a diagnosis - VIW is preceded by a coryzal illness, and has no atopic history

Answer 48

The symptoms of viral induzed wheeze are due to bronchospasm, whereas in bronchiolitis there is diameter narrowing but also secretions (leading to wet lungs)

Answer 49

Long and short: inflammation and infection in the bronchioles. RSV invades the nasopharyngeal epithelium and spreads to the lower airways where it causes increased mucous production, desquamation, and then bronchiolar obstruction. The net effect is pulmonary hyperinflation and atelectasis

Answer 50

In adults, when there is swelling and mucus in the airways, there is little affect on breathing due to size of the lumens. In infants, airways are small, so there is reduced air movement to and from the alveoli; airflow obstruction

Answer 51

Prematurity, congenital heart disease, immunodeficiency, other chronic lung conditions (eg, CF)

Answer 52

Coryzal symptoms, dry wheezy cough, increasing breathlessness, fever

Answer 53

Fine end-respiratory crackles, high pitched wheeze

Answer 54

Children under 2, generally under 1. In the winter/spring

Answer 55

Monthly IM injection of palivizumab (monoclonal antibody antibody). This reduces risk of hospitalisation and need for mechanical ventilation

Answer 56

- nasopharyngeal swab: immunoflourescent antibody testing for RSV binding - pulse oximetry - CXR and blood gases if respiratory failure is suspected

Answer 57

- Sleep apnoea - persistent oxygen sats <90% - inadequate oral fluid intake (below 50% of normal) - severe respiratory distress: grunting, marked chest recession, RR >70/min

Answer 58

- supportive management with O2 (via nasal cannula) - nasal suction - fluids by NG tube or IV - no evidence for use of nebulised bronchodilators, abx, or steroids - may need ventilation if distress: CPAP to maintain airways, and intubation and ventilation via endotracheal tube if indicated

Answer 59

Increase in CO2 and decrease in O2

Answer 60

Constrictive bronchiolitis; rare condition associated with permanent obstruction of the bronchioles due to chronic inflammation, leads to scar tissue formation

Answer 61

Adenovirus

Answer 62

autosomal recessive

Answer 63

Chromosome 7, mutation to CFTR gene (CF transmembrane conductance regulator). Leads to defective ion transport in exocrine glands.

Answer 64

Bloodspot screening (Guthrie card). Measures immunoreactive trypsinogen.

Answer 65

pseudomonas aeruginosa

Answer 66

- Airways: reduction in airway surface liquid, impaired ciliary function, retention of mucopurulent secretions - dysregulation of inflammation and defence against infection - pancreatic duct: blocked by thick secretions, leads to pancreatic enzyme deficieny and malabsorption - infertility

Answer 67

Meconium ileus (the meconium is thick and sticky, therefore gets stuck and obstructs the bowel). Presents as not passing meconium within 24 hours, abdominal distension and vomitingW

Answer 68

- chronic cough - thick sputum production - recurrent respiratory tract infections (pneumonia, bronchiectasis) - steatorrhoea (due to lack of fat digesting lipase enzymes) - abdominal pain and bloating - failure to thrive - older children: diabetes, delayed onset puberty

Answer 69

- nasal polyps - finger clubbing due to chronic hypoxia - crackles and wheezes on auscultation - hyperinflation of the chest

Answer 70

cyanotic heart disease, infective endocarditis, TB, IBD, liver cirrhosis, CF (conditions that cause chronic hypoxia, or have malabsorption)

Answer 71

Thick pancreatic and biliary secretions block the ducts. Leads to pancreatic exocrine insufficiency (lipase, amylase, protease). Results in maldigestion and malabsorption

Answer 72

Sweat test

Answer 73

Very hard to treat and worsens prognosis. Can be passed between CF patients

Answer 74

Tobramycin

Answer 75

S.aureus. Treatment is continuous prophylactic oral abx (fluclox)

Answer 76

hyperinflation, increased antero-posterior diameter, bronchial dilation, cysts, linear shadows, infiltrates

Answer 77

Bilateral sequential lung transplantation

Answer 78

Pulmonary function test (10-15% decrease), sputum cultures, CXR

Answer 79

CREON tablets to help digest fats in patients with pancreatic insufficiency (replaces lipase enzymes)

Answer 80

Nebulised DNase. This is an enzyme that can break down the DNA material in respiratory secretions, making them less viscous and easier to clear

Answer 81

Bordetella pertussis (gram negative bacteria)

Answer 82

1. Mild coryzal symptoms, fever, cough (1 week) 2. Severe paroxysmal cough, followed by inspiratory whoop and vomiting

Answer 83

Coughing fits so severe that the child is unable to take in any air between coughs and subsequently makes a loud whooping sounds as they forcefully suck in air after the coughing finishes (paroxysmal cough). Can cough so hard, followed by fainting, vomiting, nosebleed, or even developing a pneumothorax. During coughing, mucus normally flows from nose and mouth. Known as 100 day cough, as can persistent for a long time!

Answer 84

- offered to pregnant women - children; 2, 3, and 4 months. Then booster ~3 years

Answer 85

nasopharyngeal nasal swab for culturing organism

Answer 86

Macrolide prophylaxis

Answer 87

Azithromycin. This will reduce contagiousness (infectivity period), but doesn't alter clinical course. Other than this, supportive management

Answer 88

Until cough for 21 days, or antibiotics for 5 days. Not contagious after this

Answer 89

Parenchymal lung disorder characterised by pulmonary oedema resulting from delayed resorption and clearance of foetal alveolar fluid

Answer 90

TTN, respiratory distress syndrome, meconium aspiration, pneumothorax

Answer 91

Lack of surfactant

Answer 92

Premature, <32 weeks

Answer 93

Treatment is O2. It should resolve in a few days with resorption of lung fluid

Answer 94

Tachypnoea Tachycardia Nasal Flaring Use of accessory respiratory muscles Intercostal and subcostal recession Head retraction Inability to feed

Answer 95

Cyanosis Tiring because of increased work of breathing Reduced consciousness level Oxygen saturation <92% despite O2

Answer 96

- E-preemies with bronchopulmonary dysplasia - Haemo-dynamically significant congenital heart disease - disorders causing muscle weakness - CF - immuno deficiency

Answer 97

Predominantly inspiratory. From extra thoracic airway obstruction in the trachea and larynx

Answer 98

Predominantly expiratory from intrathoracic airway narrowing

Answer 99

- sudden onset dyspnoea - decreased breath sounds - inspiratory stridor - expiratory wheezing (focal)

Answer 100

Laryngeal and tracheal infection, eg, croup

Answer 101

Immature cartilage of the upper larynx collapses during inhalation

Answer 102

stridor; noisy breathing in an otherwise well child

Answer 103

Degree of stridor and chest retraction

Answer 104

Central cyanosis, drooling or reduced level of consciousness

Answer 105

From 24-28 weeks. Type II pneumocytes

Answer 106

Diffuse granular/'ground glass appearance'

Answer 107

Dexamethasone given to mothers at risk of premature delivery

Answer 108

- viral: bilateral consolidation - bacterial: lobar consolidation

Answer 109

10% improvement

Answer 110

Causes smooth muscle relaxation

Answer 111

Group A strep

Answer 112

Rhinovirus, Corona Virus, Parainfluenza

Answer 113

Determines probability of bacterial infection, and will benefit from ABx

Answer 114

Calculates the likelihood of strep throat and the need for antibiotic prescription

Answer 115

surfactant decreases the surface tension on the small airways and alveoli, which prevents the collapse of alveoli. Leads to increased surface tension, so increased pressure is required to maintain the alveolar shape. Atelectasis occurs throughout the lung, which reduces gas exchange. Repeated atelectasis results in an inflammatory response as the respiratory epithelium is damaged. Pulmonary oedema can develop.

Answer 116

Usually resolves over time as the larynx matures and grows and is able to support itself

Answer 117

the aryepiglottic folds are shortened, which pulls on the epiglottis and changes it shape to a characteristic “omega” shape. During inspiration, the soft tissue of the supraglottic larynx is soft and pulls across the airway and partially obscures it.

Answer 118

Pharyngeal (branchial) arches, pouches, and clefts

Answer 119

The auricle/pinna, and external acoustic meatus (ear canal)

Answer 120

Tympanic cavity (air filled section in the temporal bone), lined with a mucous membrane. Auditory ossicles + eustachian tube

Answer 121

Formed of the bony and membranous labryinth, Vestibulocochlear organs: bony labryinth, membranous labryinth, utricle and saccule, semicircular ducts, cochlear duct

Answer 122

Infection of the middle ear

Answer 123

Bacteria enters from the back of the throat via the eustachian tube

Answer 124

Viruses, streptococcus pneumoniae, h. influenzae, s.aureus

Answer 125

Younger age, male sex, smoking in the household, formula feeding, craniofacial abnormalities (downs, cleft palate)

Answer 126

As children grow bigger, the angle between the Eustachian tube and the wall of the pharynx becomes more acute, so that coughing or sneezing tends to push it shut. In small children, the less acute angle facilitates infected material being transmitted through the tube to the middle ear

Answer 127

Typically preceded by a viral upper respiratory tract infection. Ear pain (otalgia) , reduced hearing the in the affected ear, fever, irritability, coryzal symptoms. If vestibular system affected; balance + vertigo

Answer 128

Rupture of the tympanic membrane

Answer 129

Younger age, male sex, smoking in the household, formula feeding, craniofacial abnormalities (downs, cleft palate)

Answer 130

Red and bulging tympanic membrane with the loss of a normal light reflex. May see acute perforation

Answer 131

Middle ear effusion without the symptoms and signs of acute otitis media. Duration is often months, and there may be effusions

Answer 132

Mastoiditis, meningitis, brain abscess, facial nerve paralysis

Answer 133

6-12 months

Answer 134

Otitis media

Answer 135

Viral URTIs are thought to disturb the normal nasopharyngeal microbiome, allowing bacteria to infect the middle ear via the Eustachian tube

Answer 136

Bulging of the tympanic membrane, otorrhoea (discharge from the ear), decreased mobility on pneumatic otoscopy

Answer 137

It is generally a self-limiting condition that does not require an abx prescription. Analgesia can be given to relieve otalgia. Parents advised to seek further help if symptoms worsen/dont improve after 3 days

Answer 138

<2 years old and bilateral, present for more than 4 days, immunocompromised or at high risk of complications, otitis media with perforation/discharge in the canal

Answer 139

Tympanostomy ventilation tubes. They are treatment for recurrent secretory otitis media

Answer 140

Allow fluid from the middle ear to drain through the tympanic membrane to the ear canal

Answer 141

5-7 days of amoxicillin

Answer 142

Perforation of the tympanic membrane leads to chronic supprative otitis media. This is defined as perforation of the tympanic membrane with otorrhoea for >6 weeks. Leads to hearing loss.

Answer 143

Eardrum is seen as dull and retracted, often with visible fluid level

Answer 144

Usually resolves spontaneously but may cause conductive hearing loss as shown on pure tone audiometry (if >4 years) or a flat trace on tympanometry hearing testing in younger children

Answer 145

Hearing loss (can be asymptomatic other than this)

Answer 146

Tonsillo-adenelectomy

Answer 147

Effusion in the middle ear without an infection, that can occur after slowly resolving/recurrent acute otitis media. The fluid has a deadening effect on the vibrations of the eardrum and ossicles

Answer 148

Mishearing, difficulty communicating in a group, listening to the TV at high volumes. Lack of concentration, withdrawal. Impaired speech and language development, impaired school progress. Balance problems

Answer 149

Inflammation of the external ear canal (also known as Swimmer's ear)

Answer 150

It commonly presents with minimal discharge, itch and pain due to acute inflammation of the skin of the external auditory meatus.

Answer 151

Pseudomonas spp., and s. aureus

Answer 152

Infection (bacterial or fungal), seborrhoeic dermatitis, contact dermatitis

Answer 153

Topical antibiotic

Answer 154

Sensorineural and conductive

Answer 155

Congenital infection (such as rubella), preterm, hyperbilirubinaemia

Answer 156

Meningitis/encephalitis, head injury, drugs (such as aminoglycosides, furosemide), neurodegenerative disorders

Answer 157

May be profound (>95-dB hearing loss)

Answer 158

Amplification or cochlear implant if necessary

Answer 159

Otitis media with effusion (glue ear), Eustachian tube dysfunction, Wax (rarely)

Answer 160

Down Syndrome, Cleft palate, Pierre Robin Sequence, midfacial hypoplasia

Answer 161

Maximum of 60-dB hearing loss. This is often intermittent and resolves

Answer 162

Caused by a lesion in the cochlea or auditory nerve and is usually present at birth. It is irreversible, and can be of any severity

Answer 163

Middle ear

Answer 164

Impedance auditory tests, which measure the air pressure within the middle ear and the compliance of the tympanic membrane, determine if the middle ear is functioning normally.

Answer 165

Group A streptococcus (Streptococcus pyogenes). This can be effectively treated with penicillin V (phenoxymethylpenicillin).

Answer 166

In the pharynx, at the back of the throat, there is a ring of lymphoid tissue. There are six areas of lymphoid tissues, making up the adenoid, tubal tonsils, palatine tonsils and the lingual tonsil. The palatine tonsils are the ones typically infected and enlarged in tonsillitis. These are the tonsils at either side at the back of the throat.

Answer 167

A typical presentation is a child with a fever, sore throat and painful swallowing. It can also present with non-specific symptoms, particularly in younger children (fever, poor oral intake, headache, vomiting)

Answer 168

A score of 3 or more gives a 40 – 60 % probability of bacterial tonsillitis, and it is appropriate to offer antibiotics

Answer 169

Peritonsillar abscess (quinsy)

Answer 170

Sore throat, painful swallowing, fever, neck pain, referred ear pain, lymphadenopathy, trismus (unable to open mouth), hot potato voice (due to pharyngeal swelling)

Answer 171

Obvious ocular malformations, not smiling responsively by 6 weeks post-term, concerns about poor visual response (including eye contact), nystagmus, squint

Answer 172

Opacification of introcular structures, corneal abnormalities, or intraocular tumour (retinoblastoma)

Answer 173

Repetitive, involuntary, rhythmical eye movement

Answer 174

Infection of the peri-orbital skin

Answer 175

S.aureus or H.influenzae type B

Answer 176

URTI followed by a painful swollen eye

Answer 177

Medical emergency – requires prompt treatment with a 5-7 day course of IV antibiotics

Answer 178

- Systemically unwell with fever, erythema, tenderness over affected area - pain and swelling over the region

Answer 179

May develop into orbital cellulitis with evolving ocular proptosis, limited ocular movement, and decreased visual acuity. Orbital cellulitis can be life and sign threatening

Answer 180

Periorbital cellulitis: eyelid and skin infection infront of the orbital septum Orbital cellulitis: infection around the eyeball that involves tissues behind the orbital septum (anterior boundary of the orbit)

Answer 181

Surgical emergency with major complications including loss of vision, abscess formation, venous sinus thrombosis and extension to intracranial infection with subdural empyema, and meningitis

Answer 182

Gonorrhoea and Chlamydia

Answer 183

Painful or restricted eye movements, Visual impairment: reduced acuity or relative afferent pupil defect or diplopia, Proptosis, Severe headache or other features of intracranial involvement

Answer 184

A squint. This is a misalignment of the visual axes

Answer 185

Misalignment of the eyes. May only happen intermittently or when tired

Answer 186

Squint, eyes inwards

Answer 187

Squint, eyes deviated outwards

Answer 188

Squint, eyes drift or look upwards

Answer 189

Squint, eyes drifts or looks downwards

Answer 190

The eyes are straight when both eyes are open but a deviation of the visual axis can be elicited when each eye is covered. The affected eye will move when covered; eyes stopped from working together. This can develop to manifest strabismus

Answer 191

Looks like there is a squint, but there is not. This may be due to facial appearance (hooded eyes, unilateral ptosis, deepset eyes, facial asymmetry)

Answer 192

Hereditary, refractive error (eg, hypermetropia, anisometropia) unknown cause (common), secondary to loss of vision, anatomical/mechanical effects, neurological (rare), paralysis of muscles

Answer 193

Long-sightedness

Answer 194

Condition of asymmetric refraction between the two eyes (eg, different glasses prescription between eyes).

Answer 195

This is often due to one eye having a slightly different shape or size from the other causing unequal curving (astigmatism), unequal far-sightedness (hyperopia), or unequal near-sightedness (myopia)

Answer 196

Strabismus. Shine a pen-torch at the patient from 1 meter away. When they look at it, observe the reflection of the light source on their cornea. The reflection should be central and symmetrical. Deviation from the centre will indicate a squint. Make a note of the affected eye and the direction the eye deviates

Answer 197

Test for strabismus. Cover one eye and ask the patient to focus on an object in from of them. Move the cover across to the opposite eye, and watch the movement of the previously covered eye. If this moves inwards, means it had drifted outwards before when covered.

Answer 198

the inability of the ocular muscles to move the eye because of muscular paralysis. Can be sinister due to possibility of underlying space-occupying lesion

Answer 199

General inspection, Eye movements, fundoscopy (rule out retinoblastoma, cataracts), visual acuity, Hirschberg's test, cover test, ocular movements

Answer 200

The deviation of the eyes remains constant with changes in angle of gaze. Usually due to refractive error in both eyes. Glasses often corrects the squint

Answer 201

Defective visual acuity which persists after correction of the refractive error and removal of any pathology

Answer 202

When a squint occurs in childhood, before the eyes have fully established their connections with the brain, the brain copes with this misalignment by reducing the signal from the less dominant eye. This results in one eye that is used to see (dominant eye) and one eye they ignore (lazy eye). If this is not treated, the lazy eye gets and more disconnected from the brain (amblyopia)

Answer 203

- conservative: glasses, orthoptic exercises - surgery: muscle resection - botulinum toxin (inject muscle. Can also be an excellent diagnostic tool pre-operatively)

Answer 204

Medial rectus

Answer 205

Lateral rectus

Answer 206

Convex lenses

Answer 207

- hypermetropia (long sight): image is focused behind the retina - myopia (short sight): image focused in front of the retina

Answer 208

Squint, refractive errors, and obstruction to the visual pathway (eg, cataract)

Answer 209

Amblyopia may occur in squint when the brain is unable to combine the markedly differing images from each eye – the vision from the squinting eye is “switched off” to avoid double vision

Answer 210

- treat the underlying condition - early tx essential: after 7 years old, improvement is unlikely - patching the good eye for periods in the day to force the 'lazy' eye to work - wear appropriate glasses - atropine drops in the better eye to dilate the pupil and paralyse accomodation. Makes the other eye work.

Answer 211

Comitant: angle of deviation is the same in all positions of gaze, and extraocular movements are full Incomitant: angle of deivation is different in different positions of gaze. Extraocular movements are not full (worse)

Answer 212

May be due to restriction or paralysis of extraocular muscles.

Answer 213

- down and out - upper eyelid ptosis - inability to adduct, infraduct, or supraduct - dilated pupil with sluggish reaction

Answer 214

inturned eye (esotropia)

Answer 215

Retinoblastoma, congenital cataract

Answer 216

Down's Syndrome

Answer 217

Pulmonary stenosis, Right ventricular hypertrophy, Overriding aorta, Ventricular septal defect (babies with TOF need to PROVe themselves)

Answer 218

Benzodiazepine

Answer 219

EPHVO (extrahepatic portal vein obstruction)

Answer 220

Child is typically well and the presenting symptom is vomiting out blood. Blood tests are often normal, and the liver is only involved in very advanced cases.

Answer 221

Absent red eye reflex

Answer 222

Metered dose inhaler with large-volume spacer

Answer 223

They produce more hypoxia than bronchodilators with metered dose inhaler with spacer. They are also more expensive!

Answer 224

Large variety of mutated genes

Answer 225

Decreased faecal elastase

Answer 226

change in consistency and frequency of stools with enough loss of fluid and electrolytes to cause illness

Answer 227

Infective gastroenteritis. Usually presents with fever and vomiting

Answer 228

Infective gastroenteritis, non-enteric infections, food hypersensitivity reactions, NEC, drugs (eg, antibiotics), henoch-schonlein purpura, intussusception, haemolytic-uremic syndrome, pseudomembranous enterocolitis

Answer 229

Diarrhoea persisting for >14 days

Answer 230

Hydration and vital signs, pallor, abdominal tenderness, signs of associated illness

Answer 231

No tests necessary. Replace fluid and electrolyte losses with oral glucose-electrolyte based rehydration fluid

Answer 232

U&E, creatinine, FBC, blood gas, stool M,C&S/virology, tests for specific disease. Then IV fluid and electrolyte replacement.

Answer 233

Malabsorption, food hypersensitivity, chronic non-specific diarrhoea (toddler diarrhoea), excessive fluid intake, protracted infectious gastroenteritis, immuno-deficiencies, hirschsprung's disease, tumours, fabricated induced illness

Answer 234

IBD, constipation (impaction), malabsorption, IBS, chronic infections, laxative abuse, excessive fluid intake, fabricated induced illnesses

Answer 235

Post-infective gastroenteritis syndrome, lactose intolerance, cystic fibrosis, coeliac disease

Answer 236

Stool sample (microscopy for bacteria or parasites, leucocytes, fat globules, fatty acid crystals, faecal occult blood), blood (U+Es, FBC, increased CRP/ESR), radiology, breath hydrogen test, GI endoscopy, sweat/genetic testing, rectal biopsy

Answer 237

Lactose malabsorption or bacterial overgrowth

Answer 238

Chronic non-specific diarrhoea occurring from 6 months to 5 years

Answer 239

Reduced GI absorptive capacity (eg, coeliac disease), osmotic diarrhoea (lactase deficiency), inflammatory (eg, ulcerative colitis), secretory

Answer 240

With colicky intestinal pain, increased flatus, abdominal distension, loose stools with undigested food ('peas and carrots' stools). Child is otherwise well and thriving

Answer 241

Examination and investigations are normal

Answer 242

Reassurance: dietary (increased fat intake, normalise fibre intake, less milk, fruit juice and sugary drink intake. Loperamide occasionally may be necessary.

Answer 243

Voluntary defaecation in unacceptable places, including the child’s pants in older children. No organic abnormality is present – it is a symptom of an emotional disorder

Answer 244

Once organic disease or spurious diarrhoea secondary to constipation with loading are excluded, consider behavioural problems and referral to a child and adolescent psychiatrist

Answer 245

Acute, chronic, cyclic

Answer 246

Acute: discrete episode of moderate to high intensity. Most common and usually associated with an acute illness. Chronic: low-grade daily pattern, frequently with mild illness

Answer 247

severe, discrete episodes associated with pallor, lethargy +/- abdominal pain. The child is well in between episodes

Answer 248

Increased volumes, projectile, green/yellow, signs of illness

Answer 249

Intestinal malrotation with volvulus, atresia/stenosis of the duodenum, Hirschprung's

Answer 250

Hypertrophic pyloric stenosis, annular pancreas

Answer 251

Gastroenteritis, intussusception, gastroparesis, cyclic vomiting syndrome

Answer 252

appendicitis, functional dyspepsia, pregnancy, eating disorders

Answer 253

idiopathic, CNS disease, abdominal migraine, endocrine, metabolic, intermittent GI obstruction, fabricated illness

Answer 254

FBC, U&E, Creatinine, Stool for culture and virology, AXR, Surgical opinion if obstruction or acute abdomen possible, Exclude systemic disease

Answer 255

FBC, ESR/CRP, U&Es,LFTs, Helicobacter pylori serology, urinalysis, abdominal US, small bowel enema, sinus X-rays, test feed or abdominal US for pyloric stenosis, brain imaging (CNS tumour), consider urine pregnancy testing, upper GI endoscopy

Answer 256

Serum amylase, serum lipase, blood glucose, serum ammonia

Answer 257

- metabolic (potassium deficiency, alkalosis, sodium depletion) - nutritional - mechanical injuries to oesophagus and stomach (Mallory-Weiss, Boerhaave's syndrome, tears of the short gastric arteries resulting in shock) - dental (erosions and cavities) - oesophageal stricture - anaemia

Answer 258

Infrequent passage of stool associated with pain and difficulty or delay in defecation

Answer 259

If it cannot be explained by any anatomical or physiological abnormality

Answer 260

Low fibre diet, lack of mobility and exercise, poor colonic motility

Answer 261

Hirschsprung's disease, anal disease (infection, stenosis, ectopic, fissure, hypertonic sphincter), partial intestinal obstruction, food hypersensitivity, coeliac disease

Answer 262

Hypothyroidism, hypercalcaemia, neurological disease (eg, spinal disease), chronic dehydration (eg, diabetes insipidus), drugs (eg, opiates and anticholingergics), sexual abuse

Answer 263

Patients can develop a habit of not opening their bowels when they need to and ignoring the sensation of a full rectum. Over time they loose the sensation of needing to open their bowels, and they open their bowels even less frequently. They start to retain faeces in their rectum

Answer 264

Straining/infrequent stools, anal pain on defecation, fresh rectal bleeding (anal fissure), abdominal pain, anorexia, involuntary soiling (impaction), flatulence, decreased growth, abdominal distension, palpable abdominal masses, anal fissure, abnormal anal tone

Answer 265

Poor appetite that improves with the passage of large stool, waxing and waning of abdominal pain with passage of stool, anal pain

Answer 266

Congenital aganglionic megacolon

Answer 267

Anal stenosis

Answer 268

neurological or spinal cord abnormality

Answer 269

Anal fissure (Cycle of pain can then lead to chronic issues), haemorrhoids, rectal prolapse, megarectum, faecal impaction and soiling, volvulus, distress for child and family

Answer 270

Treat underlying cause, dietary improvements (high fibre), behavioural measurements (toilet training measures, such as 5 minute toilet time after meals)

Answer 271

Faecal softeners (movicol/lactulose), oral stimulant laxatives (senna, sodium picosulphate)

Answer 272

High dosage laxatives, such as movical

Answer 273

GTN cream, 2% lidocaine ointment.

Answer 274

Oral magnesium citrate or magnesium phosphate

Answer 275

GORD, peptic ulcer disease, H pylori infection, food intolerance, coeliac disease, IBD, constipation, UTI, dysmenorrhoea, pancreatitis, hepato-biliary disease

Answer 276

Involuntary weight loss, deceleration of linear growth, GI blood loss, significant vomiting, chronic severe diarrhoea, persistent right upper or lower quadrant pain, unexplained fever, family hx of IBD, abnormal or unexplained physical findings

Answer 277

GORD occurs when there is an inappropriate effortless passage of gastric contents into the oesophagus. GORD exists where reflux is repeated and severe enough to cause harm.

Answer 278

Not keeping any food down, projectile or forceful vomiting, bile stained vomit, haematemesis or melaena, abdominal distension, reduced consciousness/bulging fontanelle (meningitis/raised ICP), blood in stools, signs of infection, rash, apnoeas

Answer 279

Slow gastric emptying, liquid diet, horizontal posture, low resting lower oesophageal sphincter

Answer 280

In babies there is immaturity of the lower oesophageal sphincter, allowing stomach contents to easily reflux into the oesophagus. Most infants stop having reflux by 1 year old.

Answer 281

It is normal for a baby to reflux, especially after large feeds. However, signs of being problematic is when it is causing distress, chronic cough, hoarse cry, reluctance to feed, pneumonia, and poor weight gain

Answer 282

lower oesophageal sphincter dysfunction (eg, hiatus hernia), increased gastric pressure, external gastric pressure, gastric hypersecretion, food allergy, CNS disorder (such as cerebral palsy)

Answer 283

Apnoea, hoarseness, cough, stridor, lower respiratory disease (aspiration, pneumonia)

Answer 284

Effortless regurgitation in relationship to feeds

Answer 285

Oesphageal stricture, Barrett's oesophagus (premalignant intestinal metaplasia), faltering growth, anaemia, lower respiratory disease, Sandifer's syndrome

Answer 286

Rare condition causing brief episodes of abnormal movements associated with GORD in infants. Causes paroxysmal spasms of the head, neck, and back arching, but spares the limbs

Answer 287

Torticollis (forceful contraction of the neck muscles causing twisting of the neck), dystonia (abnormal muscle contractions causing twisting movements, arching of the back or unusual positions)

Answer 288

1. Positioning: nurse infants on head-up slope of 30 degrees. Burp regularly to help milk settle 2. Dietary: thickened milk feeds (infants), small frequent meals, avoid food before sleep + fatty foods 3. Drugs: gastric acid reducing drugs e.g. H2 receptor antagonists (ranitidine) or omeprazole (if oesophagitis) or Gaviscon 4. Surgery; if all else fails

Answer 289

H2 receptor antagonists (ranitidine), PPIs (eg, omeprazole)

Answer 290

Being under 5 and having D+V caused by gastroenteritis. They have a lack of benefit and cause side effects

Answer 291

Faecal-oral route, often contaminated water

Answer 292

Rota Virus or norovirus (most common). Also enteric adenovirus, astrovirus, CMV in immunocopromised

Answer 293

Watery diarrhoea (rarely bloody), vomiting, cramping abdominal pain, fever, dehydration, electrolyte disturbance, upper respiratory tract signs common with rotavirus

Answer 294

Give supportive rehydration orally or with a nasogastric tube, or IV glucose and electrolyte solution

Answer 295

>10% dehydration, unable to tolerate oral fluids

Answer 296

Enteric adenovirus; diarrhoea frequently goes beyond 10 days

Answer 297

Salmonella, Campylobacter jejuni, Shigella, Yersinia enterocolitica, E.coli, C.diff,, vibrio cholerae

Answer 298

Same as viral, plus malaise, dysentery (bloody and mucous diarrhoea), abdominal pain (can mimic appendicitis or IBD), tenesmus (VIRAL SIGNS: Watery diarrhoea (rarely bloody), vomiting, cramping abdominal pain, fever, dehydration, electrolyte disturbance, upper respiratory tract signs common with rotavirus)

Answer 299

Bacteraemia, secondary infections (particularly salmonella, campylobacter), Reiter's syndrome (Shigella, campylobacter), Guillian-Barre syndrome

Answer 300

Can't see, can't pee, can't climb a tree (reactive arthritis after an infection)

Answer 301

Stool +/- blood culture, stool C.diff toxin, sigmoidoscopy if IBD or colitis

Answer 302

Antibiotics are not indicated, as the duration of symptoms is not altered and may increase chronic carrier status, unless there is high risk of disseminated disease, presence of artificial implants, severe colitis, severe systemic illness, age <6mths, enteric fever, cholera or E.coli 0157

Answer 303

Erythromycin

Answer 304

Oral vancomycin or metranidazole

Answer 305

Pseudomembranous colitis

Answer 306

Haemolytic uraemic syndrome due to blood cells being destroyed. The use of antibiotics increases the risk of haemolytic uraemic syndrome, therefore antibiotics should be avoided if E. coli gastroenteritis is considered

Answer 307

Spread through inadequately cooked food. It grows well on food not immediately refrigerated after cooking. The typical food is fried rice left out at room temperature.

Answer 308

Whilst growing on food it produces a toxin called cereulide. This toxin causes abdominal cramping and vomiting within 5 hours of ingestion. When it arrives in the intestines it produces different toxins that cause a watery diarrhoea. This occurs more than 8 hours after ingestion. All of the symptoms usually resolves within 24 hours. SHORT COURSE

Answer 309

Yersinia enterocolitica

Answer 310

Older children or adults can present with right sided abdominal pain due mesenteric lymphadenitis (inflammation in the intestinal lymph nodes) and fever. This can give the impression of appendicitis.

Answer 311

Increased sodium and potassium. Commonly due to salmonella and e.coli

Answer 312

Mild (<5%), Moderate (5-10%), Severe (10%)

Answer 313

Reduced urine output and dry mucous membranes

Answer 314

Pale mottled appearance, cool extremities, tachycardia, tachypnoea, hypotension, delayed capillary refill, change in consciousness

Answer 315

Oral rehydration fluids (eg, diorlyte)

Answer 316

0.9% NaCl + 5% dextrose with 10mmol KCl

Answer 317

1st 10kg of bodyweight at 100ml/kg/day 2nd 10kg of bodyweight at 50ml/kg/day Remaining bodyweight at 20ml/kg/day

Answer 318

Sodium: 2-4 mmol/kg/day Potassium: 1-2 mmol/kg/day

Answer 319

Percentage dehydration = ([well weight(kg)-current weight (kg)]/well weight(kg)) x 100

Answer 320

Detectable clinical dehydration, but no red flag symptoms: 5% Red flag symptoms: 10%

Answer 321

When the child is in signs of shock

Answer 322

Fluid deficit (mL)= % dehydration x weight (kg) x 10

Answer 323

Crohn's disease favours the ileum, but can occur anywhere along the intestinal tract. Ulcerative colitis affects the colon only

Answer 324

Normal goblet cells, preserved glandular architecture, variable lymphocyte infiltration, granulomas present, transmural granulomatous inflammation. Skip lesions.

Answer 325

Depleted goblet cells, crypt abscesses, atrophic glandular architecture, heavy lymphocytic infiltrate, thickened muscularis mucosa

Answer 326

N: No blood or mucus E: Entire GI tract S: Skip lesions on endoscopy T: terminal ileum most affected and transmural inflammation S: Smoking is a risk factor

Answer 327

terminal ileum

Answer 328

C: continuous inflammation L: limited to colon and rectum O: only superficial mucosa affected S: smoking is protective E: excrete blood and mucous U: use aminosaliclyates P: primary sclerosing cholangitis

Answer 329

Diarrhoea, abdominal pain, weight loss/failure to thrive, systemic symptoms (fatigue, fever, malaise, anorexia)

Answer 330

Aphthous ulcers, abdominal tenderness, perianal abscess/fistula/skin tags, anal strictures, abdominal distension, RIF mass

Answer 331

finger clubbing, anaemia, erythema nodosum, pyoderma gangrenosum, arthritis, ankylosing spondylitis, eye issues (iritis, conjunctivitis, episcleritis), poor growth, delayed puberty, renal stones

Answer 332

Blood: FBC, ESR, CRP, U+E, LFT, ferritin, B12, folate. Serum serological markers (ASCA) Stool: faecal calprotectin Colonoscopy and biopsy (gold standard) Radiology

Answer 333

Mucosal cobblestone appearance, ulceration, dilatation, narrowed segments, fistula, skip lesions

Answer 334

Crohn's disease

Answer 335

Inducing remission: steroids (oral prednisolone or IV hydrocortisone). If insufficient, add immunosuppressant medication (azathioprine, methotrexate, infliximab). Maintaining remission: first line; azathioprine Surgery can treat strictures and fistulas.

Answer 336

Episodic/chronic diarrhoea, crampy abdominal discomfort, bowel frequency dependent on severity, urgency/tenesmus, systemic features. Blood and mucus in excretions

Answer 337

Ulcerative colitis

Answer 338

crypt abscesses, mucosal inflammation only, goblet cell depletion

Answer 339

Campylobacter, C.diff

Answer 340

Mucosal ulceration, haustration loss, colonic narrowing +/- shortening

Answer 341

Mild/moderate: 5-ASA (mesalazine) is 1st line. Can be used to maintain remission Severe: Prednisolone to induce remission Maintaining remission: mesalazine, azathioprine. Infliximab in severe cases Surgery

Answer 342

tender, red nodules on the shins caused by inflammation of the subcutaneous fat

Answer 343

Rapidly enlarging, painful skin ulcers

Answer 344

An enteropathy due to life long intolerance to gluten protein

Answer 345

Exposure to gluten causes immune reaction leading to inflammation in the small intestine. T-cell responses to gluten; create autoantibodies, target the small bowel causing villous atrophy and malabsorption.

Answer 346

Positive FHx, T1DM, Down Syndrome, IgA deficiency

Answer 347

Pallor, diarrhoea, steatorrhoea, anorexia, irritability, ulcers, dermatitis herpetiformis

Answer 348

Apathy, gross developmental delay, ascites, peripheral oedema, anaemia, delayed puberty, arthralgia, hypotonia, muscle wasting, specific nutritional disorders

Answer 349

A life threatening dehydration due to diarrhoea accompanying malabsorption

Answer 350

Because if they are IgA deficient they will not produce the usual tTG antibodies, and there may be a false negative

Answer 351

Serum tissue transglutaminase IgA antibody

Answer 352

Duodenal biopsy (must maintain high gluten diet or may be false negative!). PArticularly the third part of the duodenum

Answer 353

Diffuse, subtotal villous atrophy, increased intraepithelial lymphocytes, crypt hyperplasia

Answer 354

Atrophy of the intestinal villi

Answer 355

Coeliac disease

Answer 356

Itchy, blistering skin rash. Associated with coeliac disease

Answer 357

Birth defect characterised by the absence of ganglions in a segment of the bowel of an infant. Causes peristalsis

Answer 358

Trisomy 21

Answer 359

A mixed group of abdominal symptoms of which no organic cause can be found. Diagnosis of exclusion

Answer 360

After 6 months

Answer 361

Recurrent abdominal pain associated with at least 2 of: - relief by defecation - altered stool form - altered bowel frequency (constipation/diarrhoea)

Answer 362

MOST COMMON: premature birth Traumatic birth injuries leading to neurological disorders, cleft lip/palate, autism, neck and head abnormalities, low birth weight, respiratory problems, heart disease

Answer 363

Failure to grow at the expected rate (growth falls away from the standardised centiles).

Answer 364

Weight in infants/young children, height in older children

Answer 365

Not enough food being offered or taken. Often, poverty is the cause

Answer 366

Decreased appetite, inability to ingest, excessive food loss, malabsorption and impaired utilization, increased energy requirements

Answer 367

Severe vomiting (GORD, pyloric stenosis, dysmotility), diabetes mellitus

Answer 368

Cystic fibrosis, coeliac disease, cow's milk intolerance, chronic diarrhoea, inflammatory bowel disease, pancreatic insufficiency, inborn errors of metabolism, TD1M

Answer 369

Congenital heart disease, cystic fibrosis, malignancy, sepsis, hyperthyroidism, chronic infection (including UTI!!), TORCH infecitons, CKD

Answer 370

Two or more centile spaces

Answer 371

150ml/kg/day

Answer 372

Urine dipstick for UTI, and coeliac screen (anti-TTG). Also just always check the baby is being fed the right amount! Detailed food diary

Answer 373

- diets low in protein, energy, or specific nutrients - fad or vegetarian diets - diseases causing malabsorption, severe GORD, immunodeficiency, chronic infection - eating disorders

Answer 374

Because oedema can skew the child's weight to make them appear heavier than they are

Answer 375

A form of severe protein malnutrition characterised by oedema and an enlarged liver with fatty infiltrates

Answer 376

Kwashiorkor: sufficient calorie intake, insufficient protein intake Marasmus: insufficient calories, insufficient protein

Answer 377

Growth retardation, diarrhoea, apathy, anorexia, oedema, skin/hair depigmentation, abdominal distension with fatty liver

Answer 378

Hypoalbuminaemia, normo- and microcytic anaemia, low calcium + magnesium + phosphate + glucose

Answer 379

A nutritional deficiency where there is inadequate energy intake in all forms, including protein

Answer 380

Height preserved compared to weight, wasted appearance, muscle atrophy, listless, diarhoea, constipation

Answer 381

Day 10; if not, need admission to hospital for investigations

Answer 382

Re-feeding syndrome

Answer 383

Passage of fresh blood per the anus

Answer 384

The pylorus of the stomach enlarges as a result of hypertrophy of the circular muscle to produce the typical 'tumour'

Answer 385

White males

Answer 386

- projectile vomiting - constipation (dry nappies) - dehydration, malnutrition, jaundice

Answer 387

Projectile, starting in the 3rd-4th week of life. Non-bilious, but may contain some blood. Usually within an hour of feeding

Answer 388

Within the first 12 weeks

Answer 389

Test feed; baby feeds whilst the examiner palpates the baby's abdomen. Visible waves of peristalsis may be seen passing the upper abdomen. Pyloric tumour can usually be felt early in the feed or just after the baby has vomited

Answer 390

Firm, olive shaped mass, just above and to the right of the umbilicus during the test feed.

Answer 391

Ultrasound

Answer 392

Hypochloraemic, hypokalaemic metabolic alkalosis

Answer 393

Ramstedt's pyloromyotomy; involves splitting the thickened pyloric muscle

Answer 394

- rehydrate and correct the alkalosis before surgery - start IV fluids: 0.45% saline with 5% dextrose and 20mmol/L potassium chloride at 120 ml/kg/day - without feeds - monitor electrolytes and capillary blood pH

Answer 395

Failure of myentric plexus parasympathetic ganglion cells to migrate to the hindgut. Leads to an absence of co-ordinated bowel peristalsis and functional intestinal obstruction at the junction ('transition zone') between normal bowel and distal aganglionic bowel

Answer 396

Junction between the neuroanatomically normal ganglionic colon, and the aganglionic segment

Answer 397

Short segment; the transition zone is in the rectum or sigmoid (80% cases). Long segment; the entire colon is involved (20% cases).

Answer 398

Typically presents in the first few days of life. Low intestinal obstruction; failure to pass meconium, abdominal distension, bile-stained vomiting

Answer 399

Rectal biopsy (no ganglion cells in the submucosa). Can also do a abdominal xray, which will show the obstruction

Answer 400

Enterocolitis; a dramatic gastroenteritic illness characterised by abdominal distension, bloody watery diarrhoea, circulatory collapse, septicaemia. Usually associated with C.Difficile.

Answer 401

Myenteric plexus parasympathetic ganglion cells

Answer 402

When one segment of the intestine telescopes inside of another, causing an intestinal obstruction

Answer 403

Junction between the small and large intestines

Answer 404

Between 6 and 24 months

Answer 405

Spasms of colic associated with pallor, screaming, drawn up legs. Child asleep between episodes. As intestinal obstruction progresses, bile stained vomiting and rectal bleeding develops (red currant jelly stools).

Answer 406

Enlarged peyer's patch in the ileum acts as the lead point that invaginates into the distal bowel. Causes small bowel obstruction. Bowel becomes engorged, causing rectal bleeding. Following this perforation and peritonitis occurs

Answer 407

Ileocaecal

Answer 408

Viral gastroenteritis, as it causes the enlargement of peyer's patch, which acts as a lead point

Answer 409

Target sign

Answer 410

Sausage shaped abdominal mass

Answer 411

A pathological lead point, such as a polyp or Meckel's diverticulum

Answer 412

- Resuscitation: large volumes of IV fluid needed to restory perfusion - antibiotics - analgesia - pneumatic reduction (air pumped into colon to force bowel into normal shape) - if the above fails, laparotomy

Answer 413

Groupings of lymphoid follicles in the mucus membrane that lines the small intestine in the ileum

Answer 414

Intussusception

Answer 415

Ultrasound

Answer 416

Presence of the vitellointestinal duct (joins yolk sac to gut development) that usually involutes

Answer 417

2% of the population, within 2 feet of the ileocaecal valve, 2 inches in length, presentation before the age of 2, 2% are symptomatic, men twice as commonly as women, 2 types of tissue (gastric mucosa and pancreatic tissue)

Answer 418

Meckel's diverticulum

Answer 419

GI bleeding (presents as painless, fresh, and sufficient enough to cause a drop in Hb), obstruction, inflammation

Answer 420

Technetium 99 scan: has an affinity for gastric mucosa

Answer 421

Symptomatic, or narrow neck

Answer 422

Bowel obstruction that occurs when the meconium in a child's intestine is thicker and stickier than normal meconium, creating a blockage in the ileum.

Answer 423

Cystic fibrosis

Answer 424

Abdominal x-ray, shows characteristic findings of bubbly appearance with a lack of fluid air levels

Answer 425

Lack of pancreatic enzymes results in meconium that is thick and viscous causing an intraluminal obstruction in the terminal ileum. Babies then present at birth with intestinal obstruction

Answer 426

Gastrografin enema (provided no evidence of perforation); draws fluid into the bowel lumen. If not, laparomoty

Answer 427

Acute appendicitis

Answer 428

Faecolith (most common), tumour, intestinal parasites, hypertrophied lymphatic tissues

Answer 429

Begins with obstruction of the lumen of the appendix, often by a faecolith, causing vague central abdominal pain. Then full inflammatory process begins involving the full thickness of the wall. Irritation of the peritoneum results in more severe abdominal pain localised to the right iliac fossa

Answer 430

- abdominal pain; starts centrally, moves to RIF. Tender over McBurney's point - loss of appetitie - N+V - Rovsig's sign - guarding on abdominal palpation - rebound tenderness

Answer 431

Peritonitis, caused by a ruptured appendix

Answer 432

Palpation of the LIF causes pain in the RIF

Answer 433

ectopic pregnancy, ovarian cysts, mesenteric adentitis, appendix mass

Answer 434

Appendicetomy

Answer 435

- ultrasound or CT - generally clinical diagnosis; if suggested clinically but negative investigations, should do diagnostic laproscopy

Answer 436

Indirect inguinal hernia

Answer 437

Occur when there is a weak point in a cavity wall, usually affecting the muscle or fascia. This weakness allows a body organ that would normally be contained within that cavity to pass through the wall

Answer 438

Incaceration, obstruction, strangulation

Answer 439

Where a hernia cannot be reduced back into the proper position (irreducible). The bowel is then trapped in the herniated position. This can lead to obstruction and strangulation

Answer 440

Where a hernia is non-reducible, and the base of the hernia becomes so tight it cuts off the blood supply, causing ischaemia. Presents with significant pain and tenderness at the hernia site. Surgical emergency

Answer 441

Narrow; if it has a wide neck, there is less risk of complications. More contents can easily pass through, but also be easily put back

Answer 442

Where only part of the bowel wall and lumen herniate through, the other side remaining in the peritoneal cavity. If they become strangulated, progress rapidly to ischaemia + necrosis.

Answer 443

Where two different loops of bowel are contained within the hernia

Answer 444

Indirect: projects through the inguinal ring (lateral to epigastric vessels) Direct: projects through abdominal wall (medial to epigastric vessels)

Answer 445

Right, due to the later descent of the right testis

Answer 446

When an indirect hernia is reduced and pressure is applied (with two fingertips) to the deep inguinal ring (at the mid-way point from the ASIS to the pubic tubercle), the hernia will remain reduced

Answer 447

Due to fetal development. The processus vaginalis is a pouch of peritoneum that extends from the abdominal cavity through the inguinal canal. This allows the descent of testes (from abdominal cavity into scrotum). Normally, after they descend, the deep inguinal ring closes and the processus vaginalis is obliterated. However, in some patients this remains intact, leaving a tract from the abdominal contents. Bowel can herniate along this tract.

Answer 448

Weakness in the abdomen at Hesselbach's triangle. Pressure over the deep inguinal ring doesn't stop the herniation.

Answer 449

resuscitation of the child and then reduction of the hernia by taxis (gentle but sustained pressure is applied to the sac to reduce the contents). IF it cannot be reduced, emergency surgical exploration is necessary

Answer 450

Most will close spontaneously during the first few years of life, regardless of size. If it fails to close by 5 years, surgical repair can be done.

Answer 451

Afro-caribbean children

Answer 452

true diaphragmatic hernias are caused by the herniation of abdominal contents through a pathological defect in the diaphragm muscle.

Answer 453

Diaphragmatic hernia, which is postero-lateral

Answer 454

Pulmonary hypoplasia (incomplete development of the lungs), due to compression of the lung during development

Answer 455

Antenatal ultrasound

Answer 456

Antenatal: 20% survival Birth: 60% survival Coincidental: V.high

Answer 457

HH refers to herniation of the stomach into the chest through the oesophageal hiatus in the diaphragm. The lower oesophageal sphincter also moves and becomes incompetent

Answer 458

At the level of the lower oesophageal sphincter and be fixed in place. It should also be narrow to stop herniation.

Answer 459

Sliding (most common), rolling (rarer), combination

Answer 460

Indigestion, heartburn, acid reflux, reflux of food, burping, bloating, halitosis (bad breath)

Answer 461

Radiologically via barium meal

Answer 462

- conservative (management of GOR) - surgical if high risk of complications or severe symptoms; laproscopic fundoplication

Answer 463

When an infant who isn't sick or hungry cries for more than 3 hours a day, more than 3 days a week, for more than 3 weeks

Answer 464

Generally unknown. Should rule out underlying organic disease such as constipation, GOR, lactose intolerance, etc.

Answer 465

6 months of age

Answer 466

Can be IgE mediated, in which case there is a rapid reaction to cow's milk, occuring within 2 hours of ingestion due to histamine release. Non IgE can also occur, which means the reaction occurs over a few days.

Answer 467

Depends where the allergic inflammation is. Upper GI: vomiting, feeding aversion, pain Small intestine; diarrhoea, abdominal pain, failure to thrive Large intestine; diarrhoea, acute colitis with blood and mucus in stools General allergy: urticarial rash (hives), angio-oedema (facial swelling), cough or wheeze, sneezing

Answer 468

The reaction is to cow’s milk protein secreted into breast milk following maternal ingestion

Answer 469

Limit milk protein intake; in breast fed infants, this is via maternal exclusion diet, and in formula fed insants, feed via hydrolysed formula (short peptides).

Answer 470

Avoid using goat’s or sheep’s milk as a cow’s milk substitute, as 25% will also develop allergy to these milks (cross-reactivity). Similar cross-reactivity also often occurs with soya milk (use not recommended under 6mths)

Answer 471

They contain cow’s milk, but the proteins have been broken down so they no longer trigger an immune response. In severe cases, infants may require elemental formulas made of basic amino acids.

Answer 472

Often, children outgrow the condition by age 3 or earlier. Every 6 months, infants can be tried on the first step of the milk ladder (cookie/biscuit)

Answer 473

cookie -> muffin -> pancake -> cheese -> yoghurt -> pasteurised milk

Answer 474

deficiency of a protein that is responsible for controlling inflammatory cascades. Affects the lungs and liver, causing excess breakdown of tissue

Answer 475

Autosomal dominant

Answer 476

Autosomal dominant, chromosome 14

Answer 477

Cholestasis in infancy, may progress to liver failure Cirrhosis can occur in late childhood to adult. Chronic liver disease affects 25% of patients in late adulthood. Pulmonary emphysema is the commonest presentation in adulthood

Answer 478

Wilson's disease (copper deposits in the eye). Found via slit lamp

Answer 479

Disorder leading to a toxic accumulation of copper in the liver, and in other tissues such as the brain and eye

Answer 480

autosomal recessive

Answer 481

Liver problems usually arise first. Rarely it can present initially with neurological or psychiatric problems, although these typically occur with more advanced disease. Copper deposition in the liver leads to chronic hepatitis, eventually leading to cirrhosis. Copper deposition in the central nervous system can lead to neurological and psychiatric problems.

Answer 482

Serum caeruloplasmin; if low, indicates Wilson's, as little is being carried in the blood. Additionally, liver biopsy, and 24 hour urine copper assay

Answer 483

Copper chelation using either pencillamine or trientine

Answer 484

Childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent

Answer 485

When a baby has biliary atresia, bile flow from the liver to the gallbladder is blocked. This causes the bile to be trapped inside the liver, quickly causing damage and scarring of the liver cells (cirrhosis), and eventually liver failure

Answer 486

More women than men, and more african americans

Answer 487

- typically appear within the first two weeks to two months of life - jaundice - dark urine - alcoholic stools - weight loss and irritability - enlarged liver

Answer 488

Very high bilirubin pigments in the blood stream

Answer 489

Due to an immature liver, and the fragile red blood cells in newborns that break down rapidly, and release lots of bilirubin. Usually goes away within the first week- 10 days of life

Answer 490

Baby appears normal when born, develops at two or three weeks after birth

Answer 491

Dark urine is due to a build up of bilirubin in the blood, which is filtered by the kidney and removed in the urine. Acholic stools are due to there being no bile or bilirubin colouring being emptied into the intestine

Answer 492

- blood tests for LFTs - xrays: look for enlarged liver and spleen - abdominal ultrasound - liver biopsy - radioisotope scan will highlight the liver but poor excretion into the bowel

Answer 493

Where bile cannot be transported from the liver to the bowel

Answer 494

Excessive haemolysis taking place, liver unable to conjugate all excess bilirubin. High levels of unconjugated bilirubin

Answer 495

Reduced hepatocyte function, meaning bile is not conjugated properly

Answer 496

Obstruction of bile drainage

Answer 497

Diagnostic surgery; cholangiogram

Answer 498

Kasai procedure; re-establishes bile flow from the liver into the intestine

Answer 499

Inflammation of the liver that occurs only in early infancy

Answer 500

This is pathological, and often can indicate neonatal sepsis, so needs urgent investigations and management

Answer 501

- haemolytic disease of the newborn - ABO incompatability - haemorrhage - intraventricular haemorrhage - cephalo-haematoma - polycythaemia - sepsis - G6PD deficiency

Answer 502

- prematurity - breast milk jaundice - neonatal cholestasis - extrahepatic biliary atresia - endocrine disorders - Gilbert syndrome

Answer 503

- more than 14 days in full term babies - more than 21 days in premature babies

Answer 504

Kernicterus; type of brain damage. Due to bilirubin being able to cross the blood-brain barrier, and damaging the CNS.

Answer 505

Less responsive, floppy, drowsy baby with poor feeding

Answer 506

Jaundice that appears at one or two months, not gaining weight or growing normally, hepatosplenomegaly

Answer 507

CMV, rubella, HeP A/B/C. Due to being infected by the virus before birth via their mother, or shortly after birth

Answer 508

An infant with biliary atresia also has jaundice and an enlarged liver but is growing well and does not have an enlarged spleen. These symptoms, along with a liver biopsy and blood tests, are needed to distinguish biliary atresia from neonatal hepatitis

Answer 509

Liver biopsy. Will show 4 or 5 liver cells combined into a large cell that doesn't function as well as a normal liver cell (can be called giant cell hepatitis).

Answer 510

Neonatal hepatitis caused by the hepatitis A virus usually resolves itself within six months, but cases that are the result of infection with the hepatitis B or C viruses most likely will result in chronic liver disease

Answer 511

- chronic hepatitis (after viral hepatitis B or C) - biliary tree disease (eg, biliary atresia) - alpha1-antitrypsin deficiency - autoiummune hepatitis - wilson's disease - cystic fibrosis - primary sclerosing cholangitis - Budd Chiari syndrome

Answer 512

Jaundice, GI haemorrhage (due to portal hypertension and variceal bleeding), pruritus, failure to thrive, anaemia, enlarged hard liver, non-tender splenomegaly, hepatic stigmata, ascites, nutritional disorders, developmental delay

Answer 513

Due to affecting protein metabolism, which occurs in the liver (specifically deamination of AA, urea formation for removal of ammonia, plasma protein synthesis)

Answer 514

hepatocellular injury (high levels in inflammation or damage)

Answer 515

Derived from biliary epithelial cells and bones; so can be caused by cholestasis or bone disease

Answer 516

When it exceeds 50 micromol/L

Answer 517

Volvulus is a condition where the bowel twists around itself and the mesentery that it is attached to. Leads to closed-loop bowel obstruction, and can cause ischaemia and necrosis

Answer 518

Meconium ileus, Hirschsprung's disease, oesophageal atresia, duodenal atresia, intussusception, imperforate anus, malrotation of the intestines with volvulus, strangulated hernia

Answer 519

Persistent vomiting, often bilious. Abdominal pain and distention, failure to pass stools or wind, abnormal bowel sounds

Answer 520

Abdominal xray

Answer 521

Failure in embryonic development, mesentery doesn't fix, meaning predisposition to volvulus

Answer 522

Volvulus secondary to malrotation

Answer 523

Diarrhoea causes the lactase enzyme in the brush border of the colon to be sloughed off

Answer 524

Failure to stay dry. 1) enuresis alarm 2) desmopressin

Answer 525

Infection of the surface of the cartilage that lines the joint and the synovial fluid that lubricates the joint

Answer 526

boys under 2

Answer 527

S.aureus, group B streptococcus, gram negative bacilli, candida albicans

Answer 528

S.aureus, haemophilus influenzae, group A strep, s.pneumoniae, neirsseria gonorrhoeae (child abuse)

Answer 529

S.aureus, Group A strep

Answer 530

Neisseria gonorrhoeae (sexually active)

Answer 531

75% in the lower limb. Knee > hip > ankle

Answer 532

Can develop from osteomyelitis (especially in neonates due to trans-epiphyseal vessels), haematogenous spread of infection, direct inoculation

Answer 533

- infants: 50% don't have a fever and don't appear ill. Consider as a differential - older children: acute onset. Hot, red, swollen and painful joint. Refusing to weight bear, stiffness and reduced range of motion. Systemic symptoms such as fever, lethargy, sepsis

Answer 534

- GOLD standard: joint aspiration. Send aspirate for MC&S - blood: FBC, ESR, CRP, blood culture - xray of joint: normal initially, joint space narrowing and erosive changes - US: detect effusion and guide aspiration - MRI: to exclude osteomyelitis

Answer 535

If septic joint with h.influenzae. Increased incidence of meningitis

Answer 536

- medical: IV abx after aspirate taken for 3 weeks, then oral abx for 4-6 weeks - surgical: early referral to orthopaedic team - splintage - physiotherapy

Answer 537

Can destroy the joint and cause serious systemic illness. Hip joint infection has the worst prognosis; avascular necrosis of the femoral head can occur

Answer 538

infection in the bone, that can include the periosteum, medullary cavity, and cortical bone

Answer 539

The region where the epiphysis joins the diaphysis

Answer 540

Most infections are spread via the haematogenous route from a 1° degree of entry e.g. respiratory. May also enter through open fractures/open wounds

Answer 541

Open bone fracture, orthopaedic surgery, immunocompromised, sickle cell anaemia, HIV, TB, diabetes, peripheral arterial disease, chronic joint disease

Answer 542

Long bones; tibia > femur > humerus

Answer 543

Pain, limping, refusal to walk/weight bear, fever, malaise, flu-like symptoms, overlying may be tender with/without swelling

Answer 544

Blood: FBC, ESR, CRP, blood cultures Bone XRay US-guided aspiration for MC&S MRI (gold standard for diagnosis) Bone scans Open biopsy

Answer 545

Often normal in early stages. Late stages may have metaphyseal rarefaction. Destructive changes may appear in the bone after 10 days

Answer 546

Antibiotics: 1st line IV flucloxacillin, clindamycin if penicillin allergic. Surgical: drainage and debridement

Answer 547

Hip disorders

Answer 548

- FHx - Female - Left > Right - Breech presentation

Answer 549

Ortolani, and barlow

Answer 550

- Capsule laxity: increased type III collagen, maternal oestrogens - Decreased intrauterine volume: breech position, first born, oligohydramnios

Answer 551

Done to see if hip already dislocated. Hip is abducted and gentle pressure is applied to the proximal thigh from behind. Trying to relocate an already discloated femoral head back into the acetabulum. If dislocated, a palpable 'clunk' is noticed as the head slides back into place

Answer 552

Can the hip be dislocated? Gently adduct and depress femur; the vulnerable hip dislocates

Answer 553

Avascular necrosis, early oestroarthritic changes

Answer 554

Ultrasound. However, if the infant is > 4.5 months then x-ray is the first line investigation

Answer 555

Most unstable hips will spontaneously stabilise by 3-6 weeks. <6 months: Pavlik harness (maintain hip in flexed position with some hip abduction). Old children may require surgery

Answer 556

Bone sbcesses may become surrounded by thick, fibrous tissue and sclerotic bone (Brodie's abscess)

Answer 557

Degenerative condition affecting the hip joint of children, due to avascular necrosis of the femoral head, specifically the femoral epiphysis. Impaired blood supply to the femoral head causes bone infarction

Answer 558

Boys, aged 4-8 years old (P for perthes, p for primary school)

Answer 559

1. Avascular: hip appears sclerotic, minimal loss of epiphyseal height 2. Fragmentation: fissures in epiphysis, followed by severe fragmentation and loss of height 3. Remodelling: regeneration, new bone formation, head remodelling 4. healed

Answer 560

- classical 'painless limp' (though hip pain can develop later) - stiffness and reduced range of hip movement - xray changes - no hx of trauma

Answer 561

Early: widening of joint space Later: decreased femoral head size/flattening

Answer 562

X-ray. If normal and symptoms persists, technetium 99 bone scan or magnetic resonance imaging

Answer 563

The main complication is a soft and deformed femoral head, leading to early hip osteoarthritis. This leads to an artificial total hip replacement in around 5% of patients.

Answer 564

Variable. Initially is conservative, aiming to maintain a healthy position and joint alignment, reduce risk of damage or deformity to the femoral head (bed rest, crutches, analgesia). Later: physio, regular xrays, surgery in severe cases

Answer 565

Dislocation of the upper femoral epiphysis on the growth plate (displaced). Femoral neck displaces anteriorly and head remains in the acetabulum

Answer 566

Boys, aged 10-15. More common obese children

Answer 567

- hip, groin, thigh or knee pain - restricted range of hip movement - painful limp - restricted movement in the hip - minor trauma, with disproportionate levels of pain

Answer 568

Patient will prefer to keep the hip in external rotation. Will have limited movement of the hip, particularly restricted internal rotation. Limited flexion

Answer 569

- obese hypogonadal (low circulating sex hormone; poor skeletal maturation) - tall, thin, often bous, past growth spurt (over abundance of growth hormones)

Answer 570

Xray (AP and lateral views are diagnostic)

Answer 571

Aim: prevent further slippage and minimise complications - surgery: internal fixation. Typically a single cannulated screw placed in the centre of the epiphysis

Answer 572

Knock knees. When standing with knees together, medial malleoli are not touching

Answer 573

Bowed knees. When the patient stands with ankles together, knees bow

Answer 574

Spectrum of conditions primarily affecting the epiphyses, may involve the cartilage and bone. Not always due to inflammation, may be due to trauma or over-usage

Answer 575

Failure of the tibia tubercle apophysis due to repetitive traction stress from the extensor mechanism. Type of osteochondrosis

Answer 576

Wider section at each end of a long bone

Answer 577

Shaft portion of the long bone

Answer 578

Where the epiphysis meets the diaphysis. During growth, the metaphysis contains the epiphyseal plate

Answer 579

A normal developmental outgrowth of a bone which arises from a separate ossification centre, and fuses to the bone later in development. An apophysis usually does not form a direct articulation with another bone at a joint, but often forms an important insertion point for a tendon or ligament

Answer 580

The tibial tuberosity

Answer 581

Due to the tibial tuberosity being located at the epiphyseal plate. Stress from movements at the same time as growth in the epiphyseal plate results in inflammation on the tibial epiphyseal plate. Multiple avulsion fractures occur, the patella ligament pulls away small pieces of bone. Leads to growth of the tibial tuberosity, causing a visible lump below the knee. Initially tender due to inflammation, then becomes hard and non-tender

Answer 582

- Visible/hard tender lump at the tibial tuberosity - pain in the anterior aspect of knee - pain exacerbated by exercise

Answer 583

Conservative, reduce pain and inflammation. - reduce physical activity - ice - NSAIDs

Answer 584

Full avulsion fracture, where the tibial tuberosity is separated from the tibia

Answer 585

Medial epicondyle fragmentation or avulsion, and delayed closure of the growth plate. Due to repetitive injury to elbow when throwing/serving (eg, tennis)

Answer 586

Infarction of the navicular bone presenting as medial midfoot pain and a limp in young children (M>F) especially with load-bearing sports

Answer 587

Rare anatomic variant affecting lateral meniscus of the knee. Means the meniscus is thicker than norma, often oval or disc shaped. Can be more prone to injury, such as tears

Answer 588

Mostly asymptomatic. However, can present as insidious popping or snapping without a traumatic origin, which is associated with pain, giving way, effusion, quadriceps atrophy, limited range of motion, and clicking or locking

Answer 589

Xrays are often normal. MRI can show changes. Diagnostic arthroscopy can confirm the diagnosis

Answer 590

Tests for a meniscal tear

Answer 591

Joint line tenderness and bulging, effusion, positive McMurrays

Answer 592

Temporary irritaiton and inflammation in the synovial membrane of the hip joint that causes pain, limp, and sometimes refusal to weight bear

Answer 593

Transient synovitis

Answer 594

Children with transient synovitis typically do not have a fever

Answer 595

Viral URTI

Answer 596

Limp, refusal to weight bear, groin or hip pain, no/low temperature, otherwise systemically well

Answer 597

Limitations in abduction and internal rotation of the hip

Answer 598

The log roll test assesses for pathology within the hip joint, and can be used to isolate the patient's pathology to the hip as opposed to outside of the hip joint. Abnormal if pain, clicking or increased range of motion.

Answer 599

Diagnosis of exclusion to rule out serious conditions. Clinicial examination + x-ray of hip + blood tests

Answer 600

Conservative. Bedrest, no weight bearing, heat, NSAIDs

Answer 601

Autoimmune inflamation occurs in the joints

Answer 602

A diagnosis of exclusion in children <16yrs old with a history of at least 6wks of persistent arthritis

Answer 603

Joint pain, swelling, stiffness

Answer 604

Systemic JIA, polyarticular JIA, olgioarticular JIA, enthesitis related arthritis, juvenile psoriatic arthritis

Answer 605

Sepsis, malignancy, trauma

Answer 606

Subtle salmon pink rash, high swinging fevers, lymphadenopathy, weight loss, joint inflammation and pain, splenomegaly, muscle pain, pleuritis

Answer 607

Negative antinuclear antibodies and rheumatoid factors

Answer 608

Kawasaki disease, Still's disease, rheumatic fever, leukaemia

Answer 609

Macophage activation syndrome (severe activation of the immune system, with massive inflammatory response).

Answer 610

It presents with an acutely unwell child with disseminated intravascular coagulation (DIC), anaemia, thrombocytopenia, bleeding and a non-blanching rash. It is life threatening. A key investigation finding is a low ESR.

Answer 611

Idiopathic inflammatory arthritis in 5 joints or more. Tends to be symmetrical, and affect small joints of hands and feet, as well as large joints such as hips and knees. Minimal systemic symptoms

Answer 612

Seronegative for rheumatois arthritis?

Answer 613

Involves 4 joints or less, typically only affecting a single joint (monoarthritis). Typically affects larger joints

Answer 614

Girls under 6 years old

Answer 615

Anterior uveitis

Answer 616

Patients tend not to have any systemic symptoms and inflammatory makers will be normal or mildly elevated. Antinuclear antibodies are often positive, however rheumatoid factor is usually negative

Answer 617

Boys over 6

Answer 618

Seronegative spondyloarthropathy

Answer 619

MRI: can't differentiate between stress or autoimmune aetiology

Answer 620

Psoriasis, IBD, anterior uveitis

Answer 621

Tender to localised palpation of the entheses

Answer 622

Varying joint involvement; symmetrical polyarthritis of small joints, or asymmetrical of large joints. Also, plaques of psoriasis on skin, nail pittingm onchyolysis, dactylitis, enthesitis

Answer 623

NSAIDs, Steroids, DMARDS (such as methotrexate, sulfasalazine), biological therapy (Eg, TNF inhibitors such as infliximab and adalimumab)

Answer 624

Oligoarticular

Answer 625

- gradual onset - inflammatory symptoms worse after rest of inactivity - stiffness - associated rash, fever, weightloss - FHx of arthritis, psoriasis, colitis

Answer 626

Plantar fasciitis

Answer 627

Eye issues

Answer 628

several weeks of symptoms, night pain, worsening symptoms, abnormal neurology, night sweats

Answer 629

Normal or mildly elevated

Answer 630

Inflammation of the disc space. Symptoms: fever, irritability, unwilling to walk, back pain, abdo pain

Answer 631

Spinal cord is split by a central cartilaginous/bony prominence. Can surgically resect

Answer 632

When the sacrum doesn't form properly. RF: diabetic mother

Answer 633

Increased curvature of the spine in the sagittal plane, visible in the spine

Answer 634

Postural: due to poor posture, flexible, usually painless. Corrected via physio Congenital: rigid, painful, can be associated with congenital spinal abnormality, treatment via brace?

Answer 635

>45 degrees kyphosis, with >5 degrees anterior wedging at 3 sequential vertebrae (more curved)

Answer 636

A rounded, hunched back that can't be straightened. Also back pain between shoulder blades, stiffness

Answer 637

Epiphyseal plate disturbance and anterior wedging

Answer 638

Lateral curvature (more than 10 degrees) rotation of the spine without an identifiable cause

Answer 639

FHx (daughters of affected mothers particularly), Marfans, neurofibromatosis

Answer 640

Pathological and excessive extension of the secondary spinal curves. Presents with anteriorly rotated hips and excess extension of the lumbar spine

Answer 641

Thoracolumbar spine

Answer 642

Dyspnoea and restrictive lung function pattterns

Answer 643

Abdominal pain and 'tightening' or abdominal viscera

Answer 644

Inherited condition affecting collagen maturation and organisation causing brittle bones

Answer 645

Hypermobility, blue sclera, triangular face, short stature, deafness from early childhood, dental problems, bone deformities, joint and bone pain

Answer 646

Type 1 collagen

Answer 647

autosomal dominant

Answer 648

Type I: mild Type II: lethal Type III: severe deforming Type IV: intermediate

Answer 649

Initial bone healing is normal, but there is no subsequent remodelling, and the bone heals with a deformity

Answer 650

- prenatal US scan: can detect severe forms in foetus - molecular genetic testing - biochemistry: adjusted calcium, phosphate, PTH and ALP results are normal - skin biopsy: high collagen in fibroblasts - bone biopsy: histology

Answer 651

- bisphosphonates to increase bone density - vitamin D supplementation to prevent deficiency - supportive therapy - surgery: intramedullary rods

Answer 652

Metabolic bone disease characterised by inadequate mineralisation of bone and epiphyseal cartilage in the growing skeleton of children

Answer 653

Deficiency in vitamin D or calcium.

Answer 654

Hereditary hypophosphataemic rickets

Answer 655

Vitamin D deficiency (source: sunlight), nutritional (diet, malabsorption of calcium, CKS, GI bypass surgery)

Answer 656

Metaphyseal swellings (wide wrists), bowing deformities, slowing of linear growth, kyphoscoliosis, Harrison's sulcus motor delay, hypotonia, fractures, respiratory distress

Answer 657

Low phosphate (fasting), serum calcium variable, raised serum alkaline phosphate, low vitamin D

Answer 658

indentation on the chest roughly along the 6th rib

Answer 659

Serum 25-hydroxyvitamin D is the laboratory investigation for vitamin D. A result of less than 25 nmol/L establishes a diagnosis vitamin D deficiency

Answer 660

Greenstick (one side of the bone breaks, other side intact). Children have more cancellous bone, which is the spongy, highly vascular bone in the centre of long bones; they are flexible but less strong

Answer 661

Fractures at the growth plate

Answer 662

1. Paracetamol or ibuprogen 2. Morphine

Answer 663

10-20 years

Answer 664

Xray. Shows a poorly defined lesion in the bone, destruction of normal bone and a fluffy appearance. Causes 'sun-burst' appearanc, and Codman triangle. Can be associated soft tissue mass

Answer 665

Periosteal elevation seen on xray due to osteosarcoma

Answer 666

Ewing's sarcoma

Answer 667

Fixed abnormal ankle position that presents at birth: club foot

Answer 668

The manipulation of a foot towards normal position via repetitive casts in talipes. It is a way of treating talipes without surgery

Answer 669

Neonate: <140g/L 1-12 months: <100g/L 1-12 years: < 110g/L

Answer 670

TAILS. Thalassaemia, Anaemia of chronic disease, iron deficiency, lead poisoning, sideroblastic anaemia

Answer 671

Bone marrow failure syndromes, myelodysplastic syndrome, megaloblastic anaemia, drugs

Answer 672

Acute blood loss, anaemia of chronic disease, aplastic anaemia, haemolytic anaemia, hypothyroidism

Answer 673

Iron deficiency

Answer 674

Inadequate intake (common due to iron being used in growth), increased demand, malabsorption, blood loss

Answer 675

Low, but 50% is absorbed

Answer 676

Pre-term, low birth weight, multiple births

Answer 677

Most children asymptomatic until bad. Tired, young children feed slower, pallor, PICA (eating inappropriate foods), hair loss, dizziness, palpitations, headaches

Answer 678

Duodenum and jejunum.

Answer 679

Iron absorption requires acid from the stomach to keep it in its soluble ferrous (Fe2+) form. When there is less acid in the stomach, it turns to the insoluble form, therefore can affect absorption

Answer 680

As ferric ions bound to transferrin (carrier protein)

Answer 681

Means there is space on the transferrin molecules for the iron to bind

Answer 682

Inflammation (released from cells)

Answer 683

When there is a normal dip in haemoglobin around 6-9 weeks in healthy term babies.High oxygen delivery to the tissues caused by the high haemoglobin levels at birth cause negative feedback. Production of erythropoietin by the kidneys is suppressed and subsequently there is reduced production of haemoglobin by the bone marrow

Answer 684

Due to incompatibility between the rhesus antigens on the surface of red blood cells of the mother and fetus. When the mother is rhesus D negative, has a baby who is rhesus positive, mother forms anti-D antibodies (sensitisation). In second pregnancy, mother's anti-D antibodies cross the placenta, and attack baby's red blood cells (causes haemolsis)

Answer 685

immune haemolytic anaemia, such as haemolytic disease of the newborn

Answer 686

Single dose of albendazole or mebendazole

Answer 687

Megaloblastic anaemia is the result of impaired DNA synthesis preventing the cell from dividing normally. Rather than dividing it keeps growing into a large, abnormal cell. This is caused by a vitamin deficiency.

Answer 688

Koilonychia, angular chelitis, atrophic glossitis, brittle hair and nails, jaundice, bone deformities

Answer 689

May cause B12 deficiency, due to defective absorption (ileal resection, IBD, coeliac disease)

Answer 690

A high level of reticulocytes in the blood indicates active production of red blood cells to replace lost cells. This usually indicates the anaemia is due to haemolysis or blood loss.

Answer 691

Malnutrition, malabsorption, increased requirements, drugs, disorders of folate metabolism

Answer 692

Autosomal dominant conditions which result in an abnormally shaped red cells

Answer 693

Normal bio-concave disc shaped is replaced by a sphere-shaped RBC due to defect with the cytoskeleton. RBC survival is reduced as it is destroyed by the spleen

Answer 694

Hereditary spherocytosis

Answer 695

Autosomal dominant

Answer 696

Failure to thrive, jaundice, splenomgealy, aplastic crisis precipitated by parvovirus infection

Answer 697

Increased anaemia, haemolysis and jaundice, without the normal response from the bone marrow of creating new RBC. Thus there is no reticulocyte response. Often triggered by parvovirus infection

Answer 698

FHx, clinical fetures, spherocytes on blood film, raised mean corpuscular haemoglobin concentration

Answer 699

Folate supplementation, splenectomy, may need to remove gallbladder

Answer 700

Blood cells are ellipses shaped rather than spheres. Autosomal dominant

Answer 701

G6PD deficiency

Answer 702

X linked recessive

Answer 703

Infections, medications (such as anti malarials) or fava beans (broad beans)

Answer 704

G6PD enzyme is responsible for protecting cells from damage by reactive oxygen species. These are produced during normal cell metabolism and in higher quantities during stress on the cell. Particularly important in red blood cells, when G6PD lacks, leads to haemolysis in RBC. Increased stress can lead to acute haemolytic anaemia

Answer 705

Anaemia, intermittent jaundice, gall stones, splenomegaly

Answer 706

Seen in G6PD, on a blood film. They are blobs of denatured haemoglobin (inclusions) within RBC

Answer 707

G6PD enzyme assay

Answer 708

Primaquine, ciprofloxacin, nitrofurantoin, trimethoprim, sulfonylurea, sulfasalazine

Answer 709

Heinze bodies, bite, and blister cells

Answer 710

3 months after (avoid false negative)

Answer 711

Genetic defect in the protein chains that make up haemoglobin (either in the alpha or beta chains)

Answer 712

Autosomal recessive

Answer 713

RBC are more fragile and break down more easily. This leads to varying degrees of anaemia. The bone marrow expands to produce extra red blood cells to compensate for the chronic anaemia. This causes a susceptibility to fractures and prominent features, such as a pronounced forehead and malar eminences (cheek bones)

Answer 714

Microcytic anaemia (low MCV), fatigue, pallor, jaundice, gallstones, splenomegaly, poor growth and development, pronounced forehead and malar eminences

Answer 715

FBC showing microcytic anaemia, haemoglobin electrophoresis to diagnose globin abnormalities, DNA testing

Answer 716

Occurs as a result of the faulty creation of RBCs, recurrent transfusions, increased absorption of iron in the gut in response to anaemia

Answer 717

If they are in iron overload

Answer 718

Fatigue, liver cirrhosis, infertility, impotence, heart failure, arthritis, diabetes, osteoporosis and joint pain

Answer 719

Chromosome 16

Answer 720

Monitoring FBC, monitoring compx, blood transfusions, splenectomy, bone marrow transplant (can be curative)

Answer 721

Chromosome 11

Answer 722

Major, intermedia, minor. Because the gene defect can consist of abnormal copies that retain some function, or deletion where there is no function at all.

Answer 723

Minor: carriers of abnormally functioning beta globin gene. One normal, one abnormal. Intermedia: two abnormal copies of the beta globin gene. Can be either two defective genes or one defective gene and one deletion. Major: homozygous for deletion genes

Answer 724

Combination of ineffective erythropoiesis and haemolysis in the spleen

Answer 725

Bone marrow tranplant. Monthly blood transufions done

Answer 726

Desferrioxamine; binds to iron in the blood, which is then passed out in the urine

Answer 727

Deletion of all four functional alpha globin genes of haemoglobin; most severe and fatal form of alpha thalassaemia

Answer 728

Thalassemia is a quantitative problem of too few globins synthesized, whereas sickle-cell anemia (a hemoglobinopathy) is a qualitative problem of synthesis of an incorrectly functioning globin

Answer 729

Autosomal recessive

Answer 730

Abnormal variant called haemoglobin S (HbS)

Answer 731

Autosomal recessive, chromosome 11. One abnormal copy of the gene results in sickle-cell trait. Patients with sickle-cell trait are usually asymptomatic. They are carriers of the condition. Two abnormal copies result in sickle-cell disease

Answer 732

During fetal development, at around 32-36 weeks gestation, fetal haemoglobin (HbF) production decreases, and adult haemoglobin (HbA) increases. There is a gradual transition from HbF to HbA. At birth, around half the haemoglobin is HbF, and half is HbA. By six months of age, very little HbF is produced, and red blood cells contain almost entirely HbA.

Answer 733

Sickle cell trait reduces the severity of malaria, without the burden of the disease. Selective advantage

Answer 734

Newborn blood spot. Pregnant women of high risk also offered screening

Answer 735

Anaemia, increased risk of infection, CKD, sickle cell crises, acute chest syndrome, stroke, avascular necrosis (large joints such as hip), pulmonary HTN, gallstones, priapism, reduced growth, leg ulcers

Answer 736

Spectrum of acute exacerbations caused by sickle cell disease, ranging from mild to life threatening.

Answer 737

Spontaneously, or dyue to dehydration, infection, stress, cold weather, deoxygenation (eg, high altitude)

Answer 738

Mostly supportive; hydration, analgesia, treat any infection

Answer 739

Known as painful crisis, and is the most common type of sickle cell crisis. Caused by sickle shaped red blood cells clogging capillaries, leading to distal ischaemia

Answer 740

Pain and swelling in the hands or feet, but can affect other areas. Can be associated with fever, and priapism in men

Answer 741

Painful and persistent erection due to blood being trapped in the penis. Urological emergency, treated by aspirating blood

Answer 742

Splenic sequestration crisis is caused by red blood cells blocking blood flow within the spleen. It causes an acutely enlarged and painful spleen. Blood pooling in the spleen can lead to severe anaemia and hypovolaemic shock.

Answer 743

Mostly supportive, with blood transfusion and fluid resuscitation to treat anaemia and shock. Splenectomy can prevent it, and is used in recurrent cases

Answer 744

Splenic infarction leading to hyposplenism, susceptibility to infections, particularly from encapsulated bacteria such as streptococcus pneumoniae and H.influenzae

Answer 745

The spleen is crucial to the host response to infection by clearing polysaccharide-encapsulated bacteria. This response involves the clearing of pathogens from the bloodstream as well as the rapid production of specific antigens

Answer 746

Aplastic crisis describes a temporary absence of the creation of new red blood cells. It is usually triggered by infection with parvovirus B19.

Answer 747

Occurs when the vessels supplying the lungs become clogged with RBCs

Answer 748

Vaso-occlusive crisis, fat embolism, or infection

Answer 749

Fever, shortness of breath, chest pain, cough, hypoxia

Answer 750

pulmonary infiltrates

Answer 751

Analgesia, hydration, antibiotics/antivirals for infection, blood transfusion, incentive spirometry (encourages effective and deep breathing), respiratory support

Answer 752

Avoid crises triggers, up to date vaccinations, antibiotic prophylaxis, hydroxycarbamide, crizanlizumab, blood transfusions, bone marrow transplant

Answer 753

penicillin

Answer 754

Hydroxycarbamide works by stimulating the production of fetal haemoglobin (HbF). Fetal haemoglobin does not lead to the sickling of red blood cells (unlike HbS). It reduces the frequency of vaso-occlusive crises, improves anaemia and may extend lifespan.

Answer 755

Monoclonal antibody that targets P-selection, which is an adhesion molecule on endothelial cells. Stops RBC sticking to blood vessel walls, reduces vaso-occlusive crises

Answer 756

Hydroxycarbamide, crizanlizumab

Answer 757

haemoglobin electrophoresis

Answer 758

Inadequate EPO production, reduced RBC lifespan, frequent blood sampling whilst in hospital, iron and folic acid deficiency

Answer 759

Most severe complication of haemolytic disease of the newborn. Causes the red blood cells to break down, and fetus gets very oedematous due to liver focusing on RBC production, so albumin falls

Answer 760

Rh-ve mother, Rh+ve baby

Answer 761

anti-D IgG antibodies

Answer 762

- test for D antibodies in Rh-ve mothers at booking - can give anti-D to non-sensitised Rh-ve mothers at 28 and 34 weeks - anti-D prophylaxis

Answer 763

anti-d immunoglobulin

Answer 764

either single dose at 28 weeks, or double dose at 28 and 34

Answer 765

Blood test used during pregnancy to screen maternal blood for the presence of red blood cells. Assess the size of fetomaternal haemorrhage, so can see how much fetal blood has entered the maternal circulation. Helps to work out how much anti-D immunoglobulin to give

Answer 766

- delivery of Rh+ve infant (live or still born) - any termination of pregnancy - miscarriage if > 12 weeks - ectopic pregnancy - antepartum haemorrhage - amniocentesis, chorionic villus sampling, fetal blood sampling Given at these times as sensitisation could occur

Answer 767

hydrops foetalis, early jaundice, kernicterus, hepatosplenomegaly, coagulopathy, thrombocytopenia, leucopenia

Answer 768

inherited blood disorder associated with a progressive deficiency of all bone marrow production of blood cells + platelets (pancytopenia) --> leads to aplastic anaemia

Answer 769

Deficiency of all three cellular components of the blood

Answer 770

Anaemia secondary to the failure of erythropoiesis

Answer 771

Failure of all three blood lines (RBC, WBC, platelets); pancytopenia

Answer 772

- normochromic, normocytic anaemia - leukopenia - thrombocytopenia - can be presenting feature of ALL or AML

Answer 773

- idiopathic - cognenital (eg, fanconi) - drugs: cytotoxics, sulphonamides, phenytoin - toxins - infections: parvovirus, hepatitis - radiation

Answer 774

cytotoxics, sulphonamides, phenytoin

Answer 775

Parvovirus B19

Answer 776

- low reticulocytes despite low Hb - normal bilirubin - negative direct antiglobulin test (Coombs test) - absent red cell precursors on bone marrow examination

Answer 777

- anaemia due to reduced RBC - infections due to reduced WBC - bruising (minimal trauma) and bleeding due to thrombocytopneia - symptoms and signs of anaemia

Answer 778

- support the blood count - bone marrow transplant - immunosuppression

Answer 779

fanconi anaemia

Answer 780

- typically presents aged 4-10 years - bruising and purpura or insidious onset anaemia - congenital abnormalities

Answer 781

autosomal recessive

Answer 782

High risk of death from bone marrow failure, transformation to acute leukaemia, or general pancytopenia complications

Answer 783

condition than can cause bone marrow failure and signs of pancreatic exocrine failure and skeletal abnormalities

Answer 784

Age of onset, FHx, bleeding hx, pattern of bleeding

Answer 785

When a toddler is starting to walk

Answer 786

haemophilia

Answer 787

Platelet disorders or von Willebran disease

Answer 788

X-linked recessive

Answer 789

Haemophilia A

Answer 790

Factor VIII

Answer 791

Deficiency of factors that activate factor X in the clotting cascade. Leads to impaired clotting

Answer 792

Haemoarthroses (bleeding into joints, can lead to crippling arthritis), intracranial bleeds (following minor head trama), prolonged bleeding after surgery or trauma

Answer 793

In the first year of life when children start to crawl/walk and fall over

Answer 794

Increased APTT and decreased factor VIII assay

Answer 795

PT is a test of the extrinsic coagulation pathway (also called the tissue factor pathway), which includes factors VII, X, V, and II. APTT tests the intrinsic pathway (also called the amplification pathway or contact system), which includes factors XII, XI, IX, VIII, X, V, and II

Answer 796

Avoid NSAIDs and IM injections (including vitamin K at birth), give prophylaxsis (IV recombinant factor VIII or IX).

Answer 797

Desmopressin. Can shorten APTT and bleeding time

Answer 798

a bleeding diathesis causing big mucosal bleeds in males and females caused by suddenly appearing autoantibodies that interfere with factor VIII

Answer 799

Due to decreased synthesis of clotting factors, decreased absorption of vitamin K, abnormalities of platelet function

Answer 800

10,9,7,2 (1972)

Answer 801

- antibodies can form to factor VIII or IX; reduces or inhibits effect of treatment - transfusion transmitted infections - issues with vascular access

Answer 802

Prolonged APTT. Normal bleeding time, thrombin time, prothrombin time

Answer 803

Autosomal dominant.

Answer 804

Roles: facilitates platelet adhesion to damaged endothelium, acts as a carrier protein for factor VIII (protects from inactivation and clearance). Thus, lack of vWF leads to bleeding tendency due to VIII deficiency AND failure of platelet adhesion

Answer 805

Type 1: most common. Mildest Type 3: rarest. most severe (recessive).

Answer 806

In von willebrand disease, spontaneous soft tissue bleeding such as large haematoma and hemarthroses are uncommon

Answer 807

Bruising, excessive prolonged bleeding after surgery, mucosal bleeding such as epistaxis and menorrhagia

Answer 808

Prolonged bleeding time, APTT prolonged, reduced factor VIII

Answer 809

Desmopressin: causes secretion of factor VIII and vWF into the plasma. TXA for mild bleeding

Answer 810

can cause hyponatraemia due to water retention leading to seizures

Answer 811

Liver disease, ITP, DIC, haemorrhagic disease of the newborn due to vitamin K deficiency

Answer 812

haemarthrosis, muscle haematoma, prolonged bleeding after surgery

Answer 813

Petechiae, bruising, contact bleeding, menorrhagia

Answer 814

Low platelet count

Answer 815

Mild: low risk of bleeding Moderate: risk of excessive bleeding Severe: risk of spontaneous bleeding

Answer 816

Immune mediated reduction in platelet count due to the production of IgG antibodies that destroy the platelets

Answer 817

Petechiae are pin-prick spots (around 1mm) of bleeding under the skin. Purpura are larger (3 – 10mm) spots of bleeding under the skin. When a large area of blood is collected (more than 10 mm), this is called ecchymoses. These are all non-blanching lesions.

Answer 818

Bleeding (gums, epistaxis, menorrhagia), bruising, petechial or purpuric rash

Answer 819

Children under 10, with a history of a recent viral illness.

Answer 820

- FBC: isolated thrombocytopenia - blood film - bone marrow examinations only required if atypical features

Answer 821

- generally none: will resolve over time - if actively bleeding or severe: Prednisolone, IV immunoglobulins, blood transfusions and platelet transufsions (temporarily work until theyre destroyed by antibodies) - avoid contact sports

Answer 822

Low platelet count remains after 6 months (rare)

Answer 823

When all spots have crusted over

Answer 824

48 hours from the last episode

Answer 825

When lesions are crusted and healed or 48 hours after commencing antibiotics

Answer 826

4 days from the onset of the rash

Answer 827

5 days from the onset of swelling

Answer 828

24 hours after commencing antibiotics

Answer 829

48 hours after antibiotics commenced

Answer 830

A condition that results from aspirin treatment of viral infections in children. Leads to compromised liver function.

Answer 831

Abnormal liver function tests, vomiting, encephalopathy (slurred speech, lethargy, coma, potentially death)

Answer 832

Medium sized vessel vasculitis

Answer 833

Fever lasting longer than 5 days. At least 4 of the following: conjunctivitis, bright red + cracked lips, strawberry tongue, cervical lymphadenopathy, red palms of hands and soles of feet, which then peel

Answer 834

Desquamating rash

Answer 835

Under 5 years old, boys, asian children

Answer 836

Coronary artery aneurysm

Answer 837

Kawasaki disease

Answer 838

- FBC: anaemia, leukocytosis, thrombocytosis - LFTs: hypoalbuminaemia, elevated liver enzymes - raised inflammatory markers - ECHO: coronary artery pathology

Answer 839

Trick question! clinical diagnosis, no one single diagnostic test

Answer 840

- high dose aspirin (reduce risk of thrombosis) - IV immunoglobulins (reduce risk of coronary artery aneursms)

Answer 841

Echocardiograms

Answer 842

- MSK: arthralgia and arthritis - CNS: aseptic meningitis, sensorineural hearing loss - GI: diarrhoea and vomiting - cardiac: congestive heart failure, myocarditis, pericardial effusion, arrhythmias - coronary aneurysms

Answer 843

Around 7-21 days post onset of fever

Answer 844

Measles, Group A streptococcus, rubella, coxsackie virus, parvovirus, human herpes virus 6

Answer 845

A rash that 'bursts forth or blooms' towards the end of incubating an infection. Widespread, symmetrically distributed. Red, discrete, or confluent macules or papules

Answer 846

Macules: red/pink discrete, flat areas that blanch on pressure Papules: small, raised lesions that blanch on pressure

Answer 847

Small, raised lesions that contain clear fluid

Answer 848

Large raised lesions containing clear fluid or pus

Answer 849

Measles virus

Answer 850

Koplik spots (greyish white spots on the buccal mucosa)

Answer 851

2 days after the fever

Answer 852

10-12 days

Answer 853

Rash starts on the face, classically behind the ears, 3-5 days after the fever. Then spreads to the rest of the body

Answer 854

Erythematous macular rash (flat)

Answer 855

4 days before to 4 days after the rash

Answer 856

3C's: cough, coryza, conjunctivitis

Answer 857

measles RNA on oral fluid specimen

Answer 858

10-14 days

Answer 859

Mostly supportive, contact public health, treat secondary infections, give MMR vaccine if they don't already have it

Answer 860

- otitis media: most common complication - pneumonia: most common cause of death - encephalititis

Answer 861

Group A haemolytic Streptococci (usually S.pyogenes)

Answer 862

Children 2-6 years, peak incidence at 4 years

Answer 863

Fever, malaise, sore throat, strawberry tongue, rash (spares palms and soles)

Answer 864

Fine, puntate erythema, appears first on the torso. Spares palms and soles. Rash often more obvious in flexures. Sandpaper texture.

Answer 865

Throat swab

Answer 866

Oral Penicillin for 10 days

Answer 867

- otitis media - rheumatic fever - acute glomerulonephritis

Answer 868

The rubella virus

Answer 869

2- 3 weeks

Answer 870

7 days before the rash to 7 days after the rash

Answer 871

Erythematous macular rash, starting on the face and spreading to the rest of the body

Answer 872

Lymphadenopathy, mild fever, joint pain, sore throat

Answer 873

In the first 20 weeks, can lead to congenital rubella syndrome: triad of deafness, blindness, congenital heart disease

Answer 874

Non specific viral rash

Answer 875

Intestinal viruses of the picornaviridae family (coxsackie A16 and enterovirus 71)

Answer 876

Mild systemic upset (sore throat, fever), oral ulcers, vesicles on palms +soles of feet (blister like)

Answer 877

Viral cultures, PCR

Answer 878

Parvovirus B19: also known as slapped cheek syndrome, and erythema infectiosum

Answer 879

Parvovirus B19

Answer 880

- mild fever, coryza, non specific viral symptoms - rash around 5 days: slapped cheeks - can then spread to limbs, sparing palms and soles; raised and itchy

Answer 881

Sunlight, heat, exercise, stress. Can worsen for many months after illness

Answer 882

Reduces erythropoiesis; doesn't affect most patients, but in SCA patients could cause severe aplastic crisis.

Answer 883

Cause severe foetal anaemia, can precipitate hydrops foetalis and subsequent miscarriage

Answer 884

Human herpesvirus 6. Rarely HHV-7

Answer 885

- 3-5 day of very high fever (up to 40 degrees), and mild systemic symptoms. Can have cough and diarrhoea. Fever suddenly dissappears - when fever settles, rash appears for 1-2 days on arms, legs, trunk, face

Answer 886

Mild erythematous macular rash, not itchy

Answer 887

Febrile seizures (most common cause)

Answer 888

Papular enanthem on the uvula and soft palate. Seen in roseola infantum

Paediatrics: NOTES Flashcards

(954 cards)