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Phase 3A > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

What is the aetiology of pneumonia in children?

A

Viruses account for 14-35% of CAP in childhood, in 20-60% of children a pathogen is not found.
Neonates: Group B strep, E. coli, Klebsiella and Staph. aureus
Infants: Strep. pneumoniae, Chlamydia
School age: Strep. pneumoniae, Staph. aureus, Group A strep, Bordetella pertussis, M. pneumoniae

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2
Q

Which children are at risk of pneumonia?

A 
Congenital lung cysts
Chronic lung disease 
Immunodeficiency 
Cystic fibrosis 
Sickle cell disease 
Tracheostomy in situ
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3
Q

What are the symptoms of pneumonia?

A 
Recent URTI
Pleuritic chest pain or abdominal pain 
Temperature (>/=38.5)
SOB
Cough
Sputum production in older children (>7y)
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4
Q

What are the signs of pneumonia?

A 
Signs of respiratory distress
Desaturation 
Cyanosis 
Decreased breath sounds 
Dullness to percussion 
Tactile vocal fremitus 
Bronchial breathing
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5
Q

What investigations would you perform for pneumonia?

A 
Sputum sample
Nasopharyngeal aspirate 
Bloods and blood culture
CXR
Pleural fluid sample 
Viral titres
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6
Q

What is the management for pneumonia?

A

<5y: Amoxicillin is 1st line. Co-amoxiclav or cefaclor for typical pneumonia. Erythromycin, clarithromycin or azithromycin for atypical pneumonia.
>5y: Amoxicillin but consider a macrolide if mycoplasma or chlamydia is suspected. If S. aureus is suspected consider a macrolide or flucloxacillin with amoxicillin

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7
Q

What is the aetiology of Croup?

A

Mucosal inflammation affecting anywhere from the nose to the lower airway. It is commonly due to parainfluenza, influenza and RSV in children aged 6m-6y. In spasmodic or recurrent croup there is a barking cough and hyperactive upper airways with no apparent respiratory tract symptoms.

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8
Q

What are the symptoms of croup?

A 
Been unwell for days
Coryza
Barking cough
Able to drink 
Mouth is closed 
Fever <38.5
Hoarse voice
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9
Q

What are the signs of croup?

A 
DO NOT EXAMINE THE THROAT!!
Stridor (rasping) 
Subcostal recession 
High RR
High HR
Drowsy
Tired 
Exhausted
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10
Q

What investigations would you perform for croup?

A

None, it is a clinical diagnosis

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11
Q

What is the management for croup?

A

Children with mild illness can be managed at home. Advise parents if there is recession and stridor at rest then they will need to return to hospital. Infants <12m need closer attention.
Moist or humidified air.
Steroids: oral prednisolone (2mg/kg/day for 3 days), oral dexamethasone (0.15mg/kg stat dose) or nebulised budesonide (2mg stat dose)
Nebulised adrenaline

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12
Q

What is the aetiology of epiglottitis?

A

It is a life-threatening swelling of the epiglottis and septicaemia due to Haemophilus influenzae type B infection

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13
Q

What are the symptoms of epiglottitis?

A 
Sore throat
Painful swallowing
Drooling
Muffled voice 
Fever
Ear pain 
Cervical lymphadenopathy 
Came on over hours 
No coryza
Slight or no cough
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14
Q

What are the signs of epiglottitis?

A 
Fever
Tachycardia
Anterior neck tenderness over the hyoid bone 
Tripod sign 
Dyspnoea 
Dysphagia
Dysphonia
Respiratory distress
Stridor
DO NOT EXAMINE THE THROAT
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15
Q

What is the differential diagnosis for epiglottitis?

A 
Pharyngitis
Laryngitis 
Inhaled foreign body
Croup
Retropharyngeal abscess
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16
Q

What investigations would you perform for epiglottitis?

A

Fibre optic laryngoscopy - gold standard. Needs to be done in a safe environment e.g. theatres so a surgical airway can be made if needed
Lateral neck XR
Throat swabs once airway secure

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17
Q

What is the management of epiglottitis?

A

IV ABx - 2nd/3rd gen cephalosporin for 7-10 days
Intubation and ICU care
Rifampicin prophylaxis to close contacts

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18
Q

What are the risk factors for asthma?

A 
History of atopy
Family history of atopy 
Inner city environment 
Socioeconomic deprivation 
Obesity 
Prematurity and low birth weight
Viral infections in early childhood
Smoking (including maternal)
Early exposure to broad spectrum antibiotics
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19
Q

What are the symptoms of asthma?

A

Cough after exercise or sometimes early in the morning, disturbing sleep
SOB
Limitation in exercise performance

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20
Q

What are the signs of asthma?

A

In children with chronic problems: barrel-shaped chest, hyperinflation, wheeze and prolonged expiration

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21
Q

What investigations would you do for asthma?

A

Spirometry:
PEFR <80% predicted for height
FEV1/FVC <80% predicted
Concave scooped shape in flow volume curve
Bronchodilator response to beta-agonist therapy (15% increase in FEV1 or PEFR)

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22
Q

What is the management for asthma?

A 
SABA: salbutamol, terbutaline
LABA: salmeterol, formoterol
SAMA: ipratroprium bromide
LAMA: tiotropium
Inhaled steroids: budesonide, beclometasone, fluticasone
Leukotriene inhibitors: montelukast
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23
Q

What is the aetiology of bronchiolitis?

A

Caused by a viral infection, most often RSV.

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24
Q

What is the epidemiology of bronchiolitis?

A

<2y but peaks between 3m and 6m

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25
Q

What are the risk factors for bronchiolitis?

A 
Prematurity <37/40
Low birth weight
Mechanical ventilation as a neonate
Age <12w
Chronic lung disease
Congenital heart disease
Neurological disease with hypotonia and pharyngeal discoordination 
Epilepsy
IDDM
Immunocompromise 
Down's syndrome
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26
Q

What is the pathophysiology of bronchiolitis?

A

RSV invades the nasopharyngeal epithelium and spreads to the lower airways which increases mucus production, desquamation and bronchiolar obstruction. Causes pulmonary hyperinflation and atelectasis.

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27
Q

What is the typical history for a patient with bronchiolitis?

A

In the winter months.
An infant with coryza, dry cough and worsening breathless. Can be wheezy, have feeding difficult or episodes of cyanosis.

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28
Q

What is found on examination of a patient with bronchiolitis?

A 
Cyanosis or pallor
Dry cough
Tachypnoea
Recession
Chest hyperinflation
Prolonged expiration
Wheezes and crackles 
Apnoea
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29
Q

What investigations would you perform for bronchiolitis?

A

Pulse oximetry
CXR
NP swab or NPA for cohorting

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30
Q

What is the management for bronchiolitis?

A

O2 to achieve SpO2 >92%
Limit oral feeds or NGT if tachypnoeic
Bronchodilators - nebulised salbutamol, ipratropium and adrenaline
Mechanical ventilation
Anti-viral therapy with ribavirin should be reserved for immunodeficient patients and those with underlying heart and lung disease

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31
Q

What is the prophylaxis for bronchiolitis?

A

Palivizumab
A monoclonal antibody to RSV.
Pre-term babies and O2-dependent infants at risk of RSV infection can receive a monthly IM injection.

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32
Q

What are the genetics involved in cystic fibrosis?

A

It is an autosomally recessive inherited disorder.
It is a defect in the CFTR gene which leads to defective ion transport in exocrine glands and thickening of respiratory mucus. The most common mutation is Delta F508 deletion

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33
Q

What are the symptoms of cystic fibrosis?

A 
Cough and wheeze
SOB
Sputum production 
Haemoptysis
Steatorrhoea
Weight loss/poor weight gain
Meconium ileus
Malabsorption
Failure to thrive
Recurrent chest infections
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34
Q

What investigations would you perform for cystic fibrosis?

A 
Sweat test
CXR
Lung function tests
Molecular genetic testing 
Sputum MC&amp;S
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35
Q

How is cystic fibrosis diagnosed?

A

It is one of the diseases screened for in the newborn screening test so it is usually found then.

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36
Q

What problems might a child with cystic fibrosis face?

A

Infancy: meconium ileus, neonatal jaundice, hypoproteinaemia and oedema
Childhood: recurrent LRTIs, bronchiectasis, poor appetite, rectal prolapse, nasal polyps, sinusitis
Adolescence: bronchiectasis, DM, cirrhosis and portal HTN, distal intestinal obstruction, pneumothorax, haemoptysis, allergic bronchopulmonary aspergillosis, male infertility, arthropathy, psychological problems

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37
Q

What is the management of cystic fibrosis?

A 
Chest physio
Antimicrobial therapy 
Annual flu vaccine
Bronchodilators 
Mucolytics
Oral azithromycin
GI management: lactulose, oral acetylcysteine solution, gastrografin
Nutrition: Creon enzymes for food, high calorie diet, salt supplementation 
Fat-soluble vitamin supplements
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38
Q

What is otitis media?

A

Infection of the middle ear associated with pain, fever and irritability.

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39
Q

What is seen on examination in otitis media?

A

A red and bulging tympanic membrane with loss of a normal light reflex. Occasionally there is acute perforation.

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40
Q

What are the causative organisms for otitis media?

A

Viruses
Pneumococcus
Group A beta-haemolytic strep
Haemophilus influenzae

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41
Q

How is otitis media treated?

A

Treatment is with broad spectrum antibiotics e.g. oral amoxicillin or co-amoxiclav and analgesia. Decongestants may also help.

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42
Q

What is secretory otitis media?

A

A middle ear effusion without the symptoms and signs of acute otitis media.
The duration often lasts months and the effusions may be serous (thin), mucoid (thick) or purulent.

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43
Q

What are the symptoms of secretory otitis media?

A

Asymptomatic but children may be noticeably inattentive or complain of hearing loss.

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44
Q

What is seen on examination in secretory otitis media?

A

The ear drum is retracted and does not move easily

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45
Q

How is secretory otitis media managed?

A

Chronic (>3m) secretory otitis media needs referral to audiology and ENT. It can be treated with myringotomy and grommets.

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46
Q

What is a squint?

A

A misalignment of the visual axes of the two eyes so that they appear to point in different directions.

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47
Q

What are the causes of squint?

A

Idiopathic
Refractive Error
Visual Loss
Ophthalmoplegia

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48
Q

What are the different types of squint?

A

Concomitant (non-paralytic) - common and usually due to refractive error in one or both eyes. Often convergent.
Non-concomitant (paralytic) - rare and usually due to cranial nerve palsy. MUST exclude an intracranial lesion.
Eso - inward
Exo - outward
Hyper - up
Hypo - down

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49
Q

What investigations would you perform for a squint?

A

Orthoptic assessment.
Gross inspection
Light reflex tests including the Bruckner test (inspection for red reflex) and cover test
Corneal reflection test

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50
Q

What is the management for a squint?

A

Correct the refractive error - wear glasses, use an eye patch on the “good eye” to “train” the weaker eye.
Eye muscle exercises
Eye muscle surgery including injection of botox in one or more extraocular muscle

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51
Q

What is acute rheumatic fever?

A

It develops in response to infection with group A beta-haemolytic strep. It is seen in children 5-15 years old and the highest incidence in those from low socioeconomic areas.

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52
Q

What are the clinical features of rheumatic fever?

A

Latent period of 2-6 weeks between onset of symptoms and a previous streptococcal infection e.g. pharyngitis.
Symptoms are non-specific but the grouping together of clinical features makes the diagnosis more likely.

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53
Q

What is the diagnostic criteria for rheumatic fever called?

A

Jones Criteria

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54
Q

What symptoms make up the Jones Criteria?

A

Major: pancarditis, polyarthritis, erythema marginartum, subcutaneous nodules, Sydenham’s chorea, emotional lability
Minor: fever, arthralgia, abnormal ECG (prolonged P-R interval), high ESR/CRP, evidence of a strep infection (e.g. raised ASO titres), history of previous rheumatic fever

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55
Q

How is the diagnosis of rheumatic fever made?

A

2 major features
OR
1 major and 2 minor features AND evidence of a previous group A Strep infection

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56
Q

What is the management for rheumatic fever?

A

Bed rest
Anti-inflammatory drugs
Corticosteroids
Diuretics/ACEi in heart failure
Antibiotics e.g. penicillin V for 10 days
Long term therapy: antibiotic prophylaxis (daily oral penicillin or monthly IM penicillin G)

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57
Q

What is the aetiology of GORD?

A 
Slow gastric emptying
Liquid diet (milk)
Horizontal posture
Low resting lower oesophageal sphincter pressure
Hiatus hernia 
Increased gastric pressure 
External gastric pressure 
Gastric hypersecretion 
Food allergy
CNS disorders e.g. cerebral palsy
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58
Q

What is the presentation of GORD?

A

GI: regurgitation, non-specific irritability, rumination, oesophagitis (heartburn, difficulty feeding and crying, painful swallowing, haematemesis), faltering growth
Resp: apnoea, hoarseness, cough, stridor, lower respiratory tract disease
Neurobehavioural: bizarre extension and lateral turning of the head, dystonic postures
Complications: oesophageal stricture, Barrett’s oesophagus, faltering growth, anaemia, lower respiratory disease

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59
Q

What investigations would you perform for GORD?

A 
OGD +/- oesophageal biopsy
24 hour oesophageal pH probe
Barium swallow with fluoroscopy 
Radioisotope "milk" scan
Oesophageal manometry
CXR
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60
Q

What is the management for GORD?

A

Conservative: Positioning, dietary changes e.g. thickened milk, no fizzy drinks
Medical: ranitidine or omeprazole, Gaviscon, prokinetic drugs e.g. domperidone, mucosal protectors e.g. sucralfate
Surgery: fundoplication

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61
Q

What are the causes of constipation in children?

A

Idiopathic: combination of low fibre diet, lack of motility and exercise, poor colonic motility.
GI: Hirschsprung’s disease, anal disease, partial intestinal obstruction, food hypersensitivity, coeliac disease
Non-GI: hypothyroid, hypercalcaemia, neurological disease (spinal disease), chronic dehydration (DI), drugs e.g. opiates and anticholinergics, sexual abuse

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62
Q

What is the presentation of constipation in children?

A 
Straining and/or infrequent stools
Anal pain on defecation 
Fresh rectal bleeding 
Abdominal pain 
Anorexia 
Involuntary soiling or spurious diarrhoea 
Flatulence 
Decreased growth
Abdominal distension 
Palpable abdominal or rectal faecal mass
Anal fissure 
Abnormal anal tone
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63
Q

What investigations would you perform for constipation in children?

A

Not usually needed. If an organic cause is suspected, bloods, AXR, bowel transit studies, rectal biopsy (for Hirschsprung’s), anal manometry and spinal imaging

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64
Q

What is the management for constipation in children?

A

Treat any underlying organic cause
Dietary: increase oral fluid and fibre intake, natural laxatives e.g. fruit juice
Behavioural measures: toilet footrests, regular 5 minute toilet time after meals, star charts, reassure parents
Treat for at least 3 months
Regular oral faecal softeners e.g. Movicol, lactulose or sodium docusate
Oral stimulant laxative e.g. senna, sodium picosulphate
Enemas e.g. micralax or phosphate enemas

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65
Q

What is failure to thrive?

A

A failure to grow at the expected rate, weight is the most sensitive indicator in infants and young children whereas height is better in the older child.

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66
Q

What are the causes of failure to thrive?

A

95% of true failure to thrive is due to not enough food being offered or taken.
Organic causes:
Decreased appetite (psychological or secondary to chronic illness)
Inability to ingest
Excessive food loss
Malabsorption
Increased energy requirements (e.g. congenital heart disease, CF, malignancy, sepsis)
Impaired utilisation

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67
Q

What is important to ask in the history for failure to thrive?

A

Pregnancy: smoking and alcohol consumption, use of medication, any illness during pregnancy
Detailed history including age of onset of FTT and timing of weaning

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68
Q

What investigations would you perform for failure to thrive?

A 
Full examination including accurate measurement of growth. 
If organic disease is possible:
Baseline investigations such as FBC, ESR/CRP, U&amp;Es, creatinine, calcium, LFTs, total protein and albumin, phosphate, immunoglobulins, coeliac screen. 
Urinalysis and MC&amp;S
IEM screen
Karyotype 
Serum lead (pica)
Sweat test
OGD and small intestine biopsy
CXR
Bone age
Skeletal survey (NAI)
Abdominal USS
CT/MRI head
Oesophageal pH monitoring
ECG 
Faecal occult blood
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69
Q

What is the management of failure to thrive?

A

If FTT resolves in a few weeks, give positive reinforcement and see in outpatients.
If FTT persists, admit to hospital for basic investigations and a supervised dietary input. Adequate growth in hospital suggests non-organic cause, explore and support family dynamics. Refer to social services if needed.
If FTT continues in hospital, investigate for an organic cause.
Provide dietetic input whatever the cause

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70
Q

What is jaundice?

A

Serum bilirubin <25-30 mmol/L. It is rare outside the neonatal period.

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71
Q

What causes unconjugated jaundice?

A

Excess bilirubin production, impaired liver uptake or conjugation.
Causes: haemolysis (spherocytosis, G6PD deficiency, sickle cell anaemia, thalassaemia, HUS) or defective bilirubin conjugation (Gilberts syndrome)

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72
Q

What causes intrahepatic cholestasis?

A 
Hepatocyte damage +/- cholestasis. Unconjugated +/- conjugated hyperbilirubinaemia. 
Causes: 
Infectious 
Toxic (drugs e.g. paracetamol OD, poisons and fungi)
Metabolic e.g. Wilson's disease
Biliary hypoplasia 
Cardiovascular 
Autoimmune hepatitis
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73
Q

What causes cholestatic/obstructive jaundice?

A 
Biliary atresia
Choledochal cyst
Candi's disease 
Primary sclerosing cholangitis
Cholelithiasis
Cholecystitis
CF
Obstructive lesion e.g. tumour
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74
Q

What investigations would you perform for jaundice?

A

Bloods: FBC, blood film, reticulocyte count, coagulation, U&Es, LFTs, bilirubin level, albumin level, TFTs, total protein, viral serology, IEM screen, ammonia level, copper studies autoantibodies
Abdominal USS
CT/MRI abdomen
Liver biopsy

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75
Q

What is the management for jaundice?

A

Remove/treat underlying cause
Correct BM if low
Phototherapy may be helpful only if jaundice has a significant unconjugated component
Treat anaemia as needed

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76
Q

What is the difference between ulcerative colitis and Crohn’s disease?

A

UC: involves the colon only, proctitis is the most common.
CD: May affect any part of the GI tract but the terminal ileum and the proximal colon is the most common.

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77
Q

What are the symptoms of IBD?

A 
Anorexia
Weight loss
Lethargy
Abdominal cramps
Diarrhoea +/- mucus/blood
Urgency and tenesmus (proctitis)
Fever
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78
Q

What are the signs of IBD?

A

GI: aphthous oral ulcers, abdominal tenderness, abdominal distension (UC > CD), RIF mass (CD), peri-anal disease
Non-GI: fever, clubbing, anaemia, erythema nodosum, pyoderma gangrenosum, arthritis, ankylosing spondylitis, iritis, conjunctivitis, episcleritis, poor growth, delayed puberty, sclerosing cholangitis, renal stones, nutrition deficiencies e.g. vit B12

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79
Q

What are the complications of IBD?

A 
"Toxic" colon dilatation
GI perforation or strictures
Pseudopolyps
Massive GI haemorrhage
Colon carcinoma
Fistulae and abscesses
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80
Q

What investigations would you order for IBD?

A

Bloods: FBC, CRP/ESR, U&Es, LFTs, albumin level, iron B12, folate and cultures, serum serological markers
Stool MC&S
Endoscopy +/- biopsy
Radiology

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81
Q

What is the management of IBD?

A

Supportive treatment: bowel rest, IV hydration, PN
Drugs: mesalazine, oral prednisolone or IV methylprednisolone
Antibiotics e.g. ciprofloxacin or metronidazole
Maintenance treatment or to treat resistant disease - immunomodulators
Dietary treatment: polymeric/elemental diets but relapse rates are high
Surgery: resection

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82
Q

What is the pathophysiology of pyloric stenosis?

A

Diffuse hypertrophy +/- hyperplasia of the smooth muscle of the pylorus. It usually occurs in infants 2-8 weeks. The pyloric muscle hypertrophy results in narrowing of the pyloric canal which can then become easily obstructed.

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83
Q

What are the symptoms of pyloric stenosis?

A

Projectile vomiting starting in the 3rd/4th week of life, vomit is ALWAYS non-bilious but may contain blood. Vomiting occurs within an hour of feeding and the baby is immediately hungry - may not be projectile in those who present early. Baby can also be constipated.

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84
Q

What are the signs of pyloric stenosis?

A

Dehydration
Malnutrition and jaundice are late signs
Hyperchloraemic, hypokalaemic metabolic alkalosis
Palpable pyloric tumour
Visible peristalsis over the stomach
Dry nappies

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85
Q

What investigations would you perform for pyloric stenosis?

A

Test feed - visible peristalsis and pyloric tumour felt
USS of the stomach
Biochemistry - low chloride, low potassium, high pH

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86
Q

What is the management of pyloric stenosis?

A

Preoperative: rehydrate and correct alkalosis before surgery.
IVI - 0.45% NaCl with 5% dextrose + 20 mmol/L KCl at 120ml/kg/day
Withhold feeds and empty stomach with an NG.
Monitor BMs and U&Es.
Surgery: Ramstedt’s pyloromyotomy - hold feed overnight post-op and then feed.
No long term sequalae

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87
Q

What is the pathophysiology of intussusception?

A

One segment of the bowel invaginates into another causing obstruction. The mesentery of the intussuscepted bowel becomes compressed, bowel wall distends and obstructs the lumen leading to ischaemia. The mucosa is sensitive to ischaemia and so sloughs off leading to “red currant jelly” stool.

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88
Q

What is the epidemiology of intussusception?

A

Male:female 3:2

Affects children 6-18 months of age

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89
Q

What are the symptoms of intussusception?

A

Spasms of colic associated with pallor, screaming and drawing up of the legs.
The child can fall asleep between episodes of colic.
Child can appear ill, listless and dehydrated.

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90
Q

What are the signs of intussusception?

A

Bilious vomiting
Palpable “sausage-shaped” mass, often in the RUQ
Absence of bowel in RLQ (Dance’s sign)
Mucoid and bloody “red currant jelly” stool
Late pyrexia

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91
Q

What investigations would you perform of intussusception?

A

Blood on PR examination
AXR
USS of the abdomen - characteristic target sign

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92
Q

What is the characteristic sign seen on USS in intussusception?

A

Target sign/doughnut sign

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93
Q

What is the management of intussusception?

A

Resuscitation, antibiotics and analgesia. NGT passes if child is vomiting
Air enema - under fluoroscopic control
Laparotomy - if pneumatic reduction fails or is contraindicated e.g. gangrenous intussusception
Recurrence rate is 10% - think of any underlying pathology e.g. Meckel diverticulum

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94
Q

What is the pathology of appendicitis?

A

Sudden inflammation of the appendix, usually initiate by obstruction of the lumen, often by a faecolith. The wall of the appendix is invaded by gut flora and becomes inflamed & infected.

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95
Q

What are the symptoms of appendicitis?

A 
Early periumbilical pain which then moves to the RIF, movement or coughing aggravates the pain. 
Nausea 
Vomiting
Anorexia
Constipation or diarrhoea
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96
Q

What are the signs of appendicitis?

A 
Low grade pyrexia
Rebound tenderness
Percussion tenderness
Guarding 
Rovsing's sign may be positive
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97
Q

What investigations would you perform for appendicitis?

A

Urinalysis is abnormal in approx 1/3 of children with acute appendicitis.
FBC - mild leukocytosis but can be normal
CRP - may be raised
USS of the abdomen - accuracy is 90%

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98
Q

What is the management of appendicitis?

A

Surgical: appendectomy - open or laparoscopic
Medical: IV Abx
Both require IVI and analgesia

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99
Q

What is the pathophysiology of Hirschsprung’s disease?

A

It is caused by a failure of the ganglion cells to migrate into the hindgut leading to an absence of the parasympathetic ganglion cells in the myenteric and submucosal plexus of the rectum, possibly extending into the colon.
It may be familial and may be associated with trisomy 21.

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100
Q

What are the symptoms of Hirschsprung’s disease?

A

Neonate: abdominal distension, failure to pass meconium within the first 48 hours of life, repeated vomiting (bilious)
Older infants and children: chronic constipation resistant to the usual treatments and requiring a daily enema, soiling and overflow diarrhoea is rare. Early satiety, abdominal discomfort and distension can lead to poor nutrition and poor weight gain.

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101
Q

What are the signs of Hirschsprung’s disease?

A

Neonate: abdominal distension - tympanic on percussion, may present with acute enterocolitis.
Older infants and children: marked abdominal distension with palpable dilated loops of colon.
PR - empty rectum and a possible forceful expulsion of faeces

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102
Q

What investigations would you perform for Hirschsprung’s disease?

A

AXR - distal intestinal obstruction
Anorectal manometry
Rectal biopsy

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103
Q

What are the two histological findings of Hirschsprung’s disease?

A

Absence of ganglion cells

Hypertrophic nerves

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104
Q

What is the management of Hirschsprung’s disease?

A

Resection of the aganglionic segment - pull-through procedure

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105
Q

What is the pathophysiology in testicular torsion?

A

Torsion of the spermatic cord causing occlusion of testicular blood vessels leading to ischaemia and loss of the testis. Germ cells are the most susceptible cell lines to ischaemia.

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106
Q

What are the symptoms of testicular torsion?

A

Sudden onset severe scrotal pain associated with nausea and vomiting. It often comes on during sport or physical activity

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107
Q

What are the signs of testicular torsion?

A

Reddening of the scrotal skin
Swollen tender testis retracted upwards
Lifting the testis over the symphysis increases pain
Absence of the cremasteric reflex - 100% sensitive and 66% specific for testicular torsion.
If prenatal, baby is born with a firm, hard, non-transilluminable scrotal mass.

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108
Q

What is the differential diagnosis of testicular torsion?

A

Torted hydatid
Epididymo-orchitis
Idiopathic scrotal oedema

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109
Q

What investigations would you perform for testicular torsion?

A

USS with integrated colour doppler of the testis

Full testicular examination

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110
Q

What is the management of testicular torsion?

A

Surgical exploration of the scrotum and bilateral orchidopexy
In a neonate and if the testis is not salvageable: orchidectomy and contralateral orchidopexy

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111
Q

What are the causes of gastroenteritis?

A

Viral: rotavirus, small round structural virus (e.g. Norwalk virus/Norovirus), enteric adenovirus, astrovirus, CMV
Bacterial: Salmonella spp., Camlyobacter jejuni, Shigella spp., Yersinia entercolitica, E. coli, C. difficile, Bacillus cereus, Vibrio cholerae

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112
Q

What are the symptoms of gastroenteritis?

A

Watery diarrhoea (viral is rarely bloody)
Vomiting
Cramping abdominal pain
Malaise (bacterial)
Tenesmus (bacterial)
Dysentery - mucus and bloody diarrhoea (bloody)

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113
Q

What are the signs of gastroenteritis?

A 
Dehydration
Electrolyte disturbance
Fever
URTI is common with rotavirus
Vomiting predominates with norovirus
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114
Q

What investigations would you perform for gastroenteritis?

A

Viral: rarely necessary, stool electron microscopy or immunoassay
Bacterial: stool +/- blood culture, CDT assay, sigmoidoscopy if IBD or colitis is suspected

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115
Q

What is the management for gastroenteritis?

A

Supportive rehydration - orally, via NGT or IV
Antibiotics are not indicated unless there is a high risk of disseminated disease, presence of artificial implants (e.g. VP shunt), severe colitis, severe systemic illness, <6m old, enteric fever, cholera or E. coli 0157

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116
Q

What are the associations in coeliac disease?

A

Positive family history
T1DM
Down’s syndrome
IgA syndrome

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117
Q

What is the presentation in coeliac disease?

A

The condition may present at any age after starting solids containing gluten.
Classic initial features: pallor, diarrhoea, pale bulky floating stool, anorexia, failure to thrive, irritability
Later signs and symptoms: apathy, gross motor, developmental delay, ascites, peripheral oedema, anaemia, delayed puberty, arthralgia, hypotonia, muscle wasting, specific nutritional disorders

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118
Q

What investigations would you perform for coeliac disease?

A

Serum tissue transglutaminase (TTG) IgA antibody and IgA level (IgA deficiency can give falsely negative serology)
Endoscopic small bowel biopsy of the third part of the duodenum - diffuse subtotal villous atrophy, increased intraepithelial lymphocytes and crypt hyperplasia

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119
Q

What is the management for coeliac disease?

A

Gluten-free diet

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120
Q

When do the testes descend into the scrotum?

A

The testes descend through the inguinal canal into the scrotum during the first trimester.
Undescended testis is seen in 3% of full-term newborn boys and 1% of boys at 1 year.

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121
Q

What are the different types of undescended testes?

A

Palpable: 80% of cases, usually at the external inguinal canal
Impalpable: 20% of cases, can be intraabdominal, inside the inguinal canal or absent

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122
Q

How is undescended testis diagnosed?

A

It is usually found in the NIPE.
Diagnostic laparoscopy is usually the preferred method to confirm intra-abdominal, inguinal or absent testis.
Examination under anaesthesia.

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123
Q

What conditions are associated with undescended testis?

A 
Prader-Willi syndrome 
Kallmann's syndrome
Laurence-Moon syndrome 
Intersexuality/congenital adrenal hyperplasia
Prune belly syndrome
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124
Q

What is the management for undescended testis?

A

Medical: hCG or GnRH - maximum success rates 20%, not usually recommended anymore
Surgical: orchidopexy

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125
Q

What is the aetiology of necrotising enterocolitis?

A

Multifactorial.
Severe intestinal necrosis is the end result of an exaggerated immune response with the immature bowel leading to inflammation and tissue injury

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126
Q

What are the predisposing factors for NEC?

A 
Prematurity
IUGR (causes chronic bowel ischaemia)
Hypoxia
Polycythaemia
Exchange transfusion 
Hyperosmolar milk feeds
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127
Q

What is the presentation of NEC?

A

It is most common in the second week after birth.
Early: non-specific illness, vomiting/bilious aspirate from the gastric tube, poor feed toleration, abdominal distension
Late: additional abdominal tenderness, blood mucus or tissue in the stool, bowel perforation, shock, DIC, multi-organ failure

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128
Q

What can be used as prophylaxis for NEC?

A

Antenatal steroids and breast milk are protective

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129
Q

What investigations would you perform for NEC?

A

FBC, U&E, creatinine studies, albumin, blood gas, blood culture, AXR, group and cross match

130
Q

What is the management for NEC?

A

Stop milk feeds for 10-14 days, NGT on free draining, Bell staging
IVABx e.g. benzylpenicillin, gentamicin and metronidazole
Systemic support
Surgery: surgical resection with primary repair or a 2 stage repair

131
Q

What conditions are associated with TOF & OA?

A

VACTERL syndrome

132
Q

What abnormalities are seen in VACTERL syndrome?

A 
Vertebral defects
Anorectal malformations
Cardiac defects
Tracheo-oesophageal defects
oEsophageal atresia +/- TOF
Renal abnormalities 
Limb deformities
133
Q

What are the symptoms of TOF/OA?

A

Respiratory distress
Choking
Feeding difficulties and frothing in the first few hours after birth
Unable to pass an NGT
H-type fistulae usually present later in infancy, usually present with a recurrent cough on feeding or recurrent chest infections

134
Q

What investigations would you perform for a TOF/OF?

A

CXR - if NGT has been passed, you can see it curling in the upper oesophageal pouch
Gap-o-gram to assess the distance between the proximal and distal parts of the oesophagus
For VACTERL: KUB USS, echocardiography, limb x-rays, USS of the spine

135
Q

What is the management for a TOF/OA?

A

Standard IV fluids
Replogle tube
Surgery: disconnection of the TOF and anastomosis of the upper and lower oesophagus through a right thoracotomy. Long gap OA may require a feeding gastrostomy and cervical oesophagostomy followed by oesophageal replacement

136
Q

When is a UTI in a child more likely to cause renal damage?

A

In pre-school children:

  • if child is <1y
  • upper UTI
  • vesico-ureteric reflux
  • delay in treatment
137
Q

What is the definition of a UTI?

A

The presence of symptoms plus the detection of a significant culture of organisms in the urine: any growth on suprapubic aspirate or >10^5 organisms/ml

138
Q

What are the clinical features of a UTI?

A

In infants it may be nonspecific: vomiting, lethargy, fever, irritability, poor feeding.
In older children: dysuria, urgency or wetting without significant systemic illness. With pyelonephritis, illness with fever and loin pain.

139
Q

What are the risk factors for a UTI?

A 
Poor urine flow 
History of a previous UTI
Recurrent fever of uncertain origin 
Antenatally diagnosed renal abnormality
FHx of vesico-ureteric reflux or renal disease
Constipation 
Dysfunctional voiding 
Abdominal mass
Evidence of a spinal lesion 
Poor growth 
High blood pressure
140
Q

What investigations would you perform for a UTI?

A

Urine sample for MC&S and urinalysis

141
Q

What is the management for a UTI?

A 
Increase fluid intake
Encourage micturition 
Antibiotics 
<3m IV Abx
>/=3m with upper UTI - oral Abx for 7-10d or IV Abx for 2-4d followed by oral Abx
>/= 3m with lower UTI - oral Abx for 3d
142
Q

What follow up do children with UTI need?

A

USS KUB
MCUG
DMSA/MAG3

143
Q

At what age should children be dry (continent of urine) by?

A

Children learn to be dry by day by approx 2 years and by night at approx 3 years. By 4 years, 75% of children are dry by day and night. It is rare to encounter a child of school age who has never had a dry night

144
Q

What are the causes of nocturnal enuresis?

A

Lack of ADH release during sleep with excess urine production
Low functional bladder capacity
Inability to wake to full bladder sensation
Interference with learning
Psychological distress
Medical conditions e.g. UTI, constipation, DM, DI
Inadvertent behaviour reinforcement
Genetic factors

145
Q

What investigations should you perform for nocturnal enuresis?

A

Examine the child to exclude physical problems and to reassure the family. Consider a urine dipstick for glucose or signs of infection, particularly if the enuresis is secondary.
Where there is normal daytime bladder function or a few nights completely dry then there can be no serious defect of the urinary tract

146
Q

What is the management of nocturnal enuresis?

A

Rewarding and encouragement e.g. Star chart

Conditioning therapy with an enuresis alarm

147
Q

What is nephrotic syndrome?

A

A combination of heavy proteinuria (PCR >200mg/mmol), hypoalbuminaemia (<25g/L), oedema and hyperlipidaemia.

148
Q

What are the different types of nephrotic syndrome?

A

Primary: congenital, infantile
Secondary:minimal change disease, focal segmental glomerulonephritis, membranous glomerulonephritis

149
Q

What are the classifications of nephrotic syndrome?

A

Steroid sensitive
Steroid dependent
Steroid resistant

150
Q

What are the clinical features of nephrotic syndrome?

A

Most children present with insidious onset of oedema which is initially periorbital but may become generalised with pitting oedema. Periorbital oedema is often most noticeable in the morning on rising. Ascites and pleural effusions may subsequently develop.

151
Q

What investigations you perform for nephrotic syndrome?

A

Full examination including height, weight and BP.
Urine sample for urinalysis, MC&S, Na and Protein-Creatinine Ratio (early morning specimen needed)
Bloods: FBC, U&E, creatinine, C3/C4, albumin, ANF, ASO titres, ANCA and Igs if mixed nephrotic/nephritic picture, lipid profile

152
Q

What is the management of nephrotic syndrome?

A

Admission, fluid restriction and prevention of hypovolaemia.
Treat hypovolaemia if present
Fluid restrict to 800-1000ml/24 hours
Diuretics if very oedematous and NO hypovolaemia - furosemide or spiro
Steroid therapy: oral prednisolone 60mg/m2/day for 4 weeks then 40mg/m2/alternate days for 4 weeks then slowly wean over 4 months
Diet - no added salt and healthy eating
Prophylactic Abx until oedema free - penicillin V
Immunise with pneumococcal vaccine

153
Q

What is nephritic syndrome?

A

Renal inflammation provoked by deposition of immune complexes causing haematuria, oligouria, hypertension and increased serum creatinine

154
Q

What is the aetiology of nephritic syndrome?

A

Best-known is post-streptococcal glomerulonephritis, it usually occurs 2-3 weeks after a beta-haemolytic strep throat. Pneumococcal pneumonia, septicaemia, glandular fever and other viral infections. Can also be seen in HSP.

155
Q

What are the clinical features of nephritic syndrome?

A 
Age 3+ (peak at 7 years) 
Sudden onset of illness 2-3 weeks after pharyngitis
Coca-cola coloured urine 
Facial oedema (especially the eyelids)
Abdo or loin pain 
Reduced urine output 
Proteinuria
Hypertension 
Deteriorating renal function 
Uraemic symptoms (anorexia, pruritis, lethargy and nausea)
156
Q

What investigations would you perform for nephritic syndrome?

A

Urine: urinalysis and MC&S (red cell casts)
Bloods: FBC, U&Es including creatinine, bicarbonate, calcium, phosphate, albumin levels, ASO titres/AntiDNAase B, complement levels (low C3, normal C4), autoantibody screen (including ANA).
KUB USS
CXR if fluid overload is suspected

157
Q

What is the management of nephritic syndrome?

A

Admit and treat any electrolyte imbalance
Supportive treatment
Treat the underlying cause e.g. 10 day course of penicillin for infection

158
Q

What is haemolytic uraemic syndrome?

A

A triad of microangiopathic haemolytic anaemia, thrombocytopenia and AKI

159
Q

What are the risk factors associated with haemolytic uraemic syndrome?

A

Rural>urban populations
June-September
Young age (6m -5y)
Contact with farm animals

160
Q

What is typical haemolytic uraemic syndrome?

A

It is associated with E.Coli O157:H7, other pathogens that induce HUS are S. pneumoniae, Shigella dysenteriae type I, HIV and Coxsackie virus

161
Q

What is atypical haemolytic uraemic syndrome?

A

Caused by exposure to certain medications e.g. ciclosporin or tacrolimus, genetic mutations in the complement pathway, systemic conditions such as SLE or cancer (or pregnancy)

162
Q

What are the clinical features of haemolytic uraemic syndrome?

A

AKI
Gut symptoms: prodrome of bloody diarrhoea, rectal prolapse, haemorrhagic colitis, bowel wall necrosis and perforation
Pancreatic symptoms: glucose intolerance/IDDM, pancreatitis
Liver jaundice
Neurological irritability - frank encephalopathy
Cardiac myocarditis (rare)

163
Q

What investigations would you perform for haemolytic uraemic syndrome?

A

Bloods: FBC & film, blood cultures, U&Es, LFTs, E. coli PCR
Stool sample for MC&S

164
Q

What is the management for haemolytic uraemic syndrome?

A 
Management is mainly supportive and directed at treating clinical features. 
Monitor electrolytes and fluid balance
Nutrition 
Blood transfusion 
Treat HTN
165
Q

What are the trigger factors for atopic eczema?

A 
Irritants
Skin infections 
Contact allergens
Extremes of temperature and humidity 
Abrasive fabrics 
Dietary factors 
Inhaled allergens 
Stress
166
Q

What are the clinical features of atopic eczema?

A

Itch leading to scratching and exacerbation of the rash. Excoriated areas become erythematous, weeping and crusted. Chronic eczema may be lichenified and dry.
Infant eczema often affects the cheeks, elbows and knees with crawling.
Childhood eczema is often flexural, also affects the wrists and ankles.
Adolescent eczema is also flexural but may also affect the head, neck, nipples, palms and soles.

167
Q

What are the causes of exacerbations of atopic eczema?

A 
Bacterial infection 
Viral infection 
Ingestion of an allergen
Contact with an irritant or allergen
Environment
Change or reduction in medication
Psychological stress
Unexplained
168
Q

What is the management of atopic eczema?

A

General measures e.g. soap avoidance, moisturising, cotton fabrics
Emollients >/= 2 times a day (ointments > creams)
Topical corticosteroids - use with care
Immunomodulators - short term use of tacrolimus or pimecrolimus
Occlusive bandages
Antibiotics or antivirals
Dietary elimination
Psychosocial support

169
Q

What is Stevens-Johnson Syndrome?

A

SJS is an immune complex-mediated hypersensitivity disorder. It ranges from mild skin and mucous membrane lesions to a severe, sometimes fatal systemic illness - toxic epidermal necrolysis (TEN).

170
Q

What is the aetiology of Stevens-Johnson Syndrome?

A

Drugs: allopurinol, carbamazepine, sulfonamides, antivirals e.g. nevirapine, abacavir, phenobarbital, phenytoin, valproic acid, lamotrigine, imidazole antifungals, NSAIDs, salicylates, sertraline, bupropion
Infections: viral e.g. HSV, EBV, HIV, coxsackie virus; bacteria e.g. Group A beta-haemolytic strep, diphtheria, typhoid, M. pneumoniae; Fungal e.g. histoplasmosis; Protozoal e.g. malaria

171
Q

What are the symptoms of Stevens-Johnson Syndrome?

A

URTI
Arthralgia
Vomiting and diarrhoea
Malaise
Mucocutaenous lesions develop suddenly and clusters of outbreaks last 2-4 weeks.
Mouth: severe oromucosal ulceration
Patients can have genitourinary and ocular symptoms

172
Q

What are the signs of Stevens-Johnson Syndrome?

A 
Fever
Tachycardia
Hypotension 
Altered level of consciousness
Seizures 
Coma 
Erythema 
Oedema 
Ulceration 
Necrolysis 
Lesions commonly affect the palms, soles, dorsum of hands and extensor surfaces
173
Q

What investigations would you perform for Stevens-Johnson Syndrome?

A

U&Es, glucose and bicarbonate for dehydration assessment
Skin biopsy
Clinical features

174
Q

What is the management for Stevens-Johnson Syndrome?

A

Identify and remove causative drug or underlying cause
Supportive: airway, IV fluids, pain relief, treat lesions in the same way as burns, treat secondary infections, immunomodulation

175
Q

What is a capillary haemangioma?

A

“stork bites”

ink macules on the upper eyelids, mid-forehead and nape of the neck are common and arise from distension of the dermal capillaries. Those on the eyelids gradually fade over 1 year, those on the neck are covered with hair.

176
Q

What is neonatal urticaria?

A

A common rash appearing at 2-3 days of age, white, pinpoint papules at the centre of an erythematous base. Fluid contains eosinophils. The rash is contained on the trunk.

177
Q

What is Mongolian blue spot?

A

Also called slate grey naevi.
A blue/black macular discolouration at the base of the spine and on the buttocks, occasionally occurs in the legs and other parts of the body. Usually in Afro-Caribbean or Asian infants. They fade slowly over the first few years. Of no significance unless misdiagnosed as bruises.

178
Q

What is a port-wine stain?

A

Also called a naevus flammeus.
Present from birth and usually grows with the infant, due to a vascular malformation of the capillaries in the dermis. If along the distribution of CN5, associated with intracranial AVM or severe lesions on the limbs with bone hypertrophy. Can use laser therapy.

179
Q

What is a strawberry naevus?

A

Also called a cavernous haemangioma.
They’re often not present at birth but appear in the first month of life and may be multiple. More common in preterm infants. Increases in size until 3-15 months old and then gradually regress. No treatment is indicated unless they interfere with vision or the wairway. Ulceration or haemorrhage may occur.

180
Q

What is Kawasaki disease?

A

An idiopathic, self-limiting systemic vasculitis, most often affects children 6m-5y.

181
Q

What are the clinical features of Kawasaki disease?

A

Fever >5 days with 4 of the 5 other features of:
Conjunctive infection (bilateral)
Mucous membrane changes (pharyngeal infection, red dry and cracked lips, strawberry tongue)
Cervical lymphadenopathy
Rash (polymorphous)
Red and oedematous palms and soles, peeling of the fingers and toes

182
Q

What investigations should you perform in Kawasaki disease?

A

Clinical diagnosis is based on the features.
Increased WCC, ESR, CRP and platelets in the acute phase.
Echo - serial echocardiography is needed to detect occult coronary artery aneurysms and disease

183
Q

What is the management for Kawasaki disease?

A

IVIg - reduces incidence of coronary artery aneurysm from 25% if untreated to 5%. 2g/kg as a single infusion over 12 hours.
Aspirin to reduce the risk of thrombosis

184
Q

What are the signs and symptoms of measles?

A

Prodrome: fever, coryza, non-purulent conjunctivitis.
Exanthematous phase: a 2nd viraemia occurs 5-7 days after the initial infection. A maculopapular rash starts on the face and lasts 6-8 days.
Infectivity: 4 days before to 4 days after the onset of the rash
Other features: generalised lymphadenopathy, anorexia, diarrhoea and fever
Incubation period: 7-18 days

185
Q

How is measles diagnosed?

A

Clinical: Koplik spots are pathognomonic and usually seen
Blood film: leukopenia and lymphopenia
LFTs: increased transaminases
Oral fluid test: measles RNA on oral fluid specimen confirms the diagnosis

186
Q

What is the management of measles?

A

Acute: generally supportive but could include antibiotics for a secondary bacterial infection e.g. otitis media

187
Q

How is measles prevented?

A

MMR vaccine at 12-18m and a preschool booster for all children

188
Q

What are some complications of measles?

A 
Approx 20% of cases 
Acute otitis media
LRTI
Encephalitis
Subacute sclerosing panencephalitis
189
Q

What are the signs and symptoms of chickenpox?

A

Prodrome: VZV is spread by respiratory droplets or direct contact with lesions. The infectious period begins 2 days before vesicles appear and end when the last vesicle crusts over.
Rash: usually starts on the head and trunk then the rest of the body. It starts as red macules then progresses to a papule then a vesicle then a pustule then crusting. Different stages of the rash are seen at the same time and heal completely within 2 weeks.
Other features: headache, anorexia, signs of URTI (sore throat, cough, coryza), fever, itching.

190
Q

How is chickenpox diagnosed?

A

Clinical: characteristic rash, its distribution and progression
Other: serology (VZV IgM), electron microscopy of vesicle fluid

191
Q

What is the management of chickenpox?

A

Symptoms: treatment of fever and itching
School exclusion: 5 days from the START of skin eruptions
Antivirals: aciclovir in severe varicella, encephalitis, pneumonia, babies and immunocompromised
VZ Ig: consider as prophylaxis
Immunisation: from age 1, not currently part of the UK schedule

192
Q

What are the risk factors of TB?

A

Close contact with a TB patient
HIV positive
Immunocompromised

193
Q

What are the clinical features of TB?

A 
Nearly 50% of infants and 90% of older children show minimal signs and symptoms of infection. 
Fever
Anorexia
Weight loss
Cough
CXR changes e.g. hilar lymphadenopathy, pleural effusion 
Erythema nodosum 
Phlyctenular conjunctivitis
194
Q

How is TB diagnosed in children?

A

Diagnosis of TB in children is hard.
Gastric washings on 3 consecutive mornings (children <8 swallow sputum).
Urine sample for MC&S.
Lymph node excision
LP for CSF MC&S
CXR - pleural effusion, lymphadenopathy, parenchymal consolidation, Ghon complex, patchy consolidation, cavitation.
If TB is suspected, a Mantoux test can be performed (history of BCG vaccination can give a false positive).

195
Q

What is the treatment for TB?

A

Triple or quadruple therapy with rifampicin, isoniazid, pyrazinamide and ethambutol for 2 months then rifampicin and isoniazid. Uncomplicated pulmonary TB or lymph node TB is treated for 6 months. Longer treatment courses are needed for TB meningitis or disseminated disease.

196
Q

What are some causative organisms of meningitis?

A

Neonate-3m: Group B strep, E. coli and other coliforms, Listeria monocytogenes
1m-6y: N. meningitides, S. pneumoniae, H. influenzae
>6y: N. meningitides, S. pneumoniae

197
Q

What is the typical history in meningitis?

A 
Fever
Headache
Photophobia
Lethargy
Poor feeding/vomiting
Irritability 
Hypotonia/"floppiness"
Drowsiness
Loss of consciousness
Seizures
198
Q

What are the typical findings on examination in meningitis?

A 
Fever
Purpuric rash (meningococcal disease)
Neck stiffness (not always present in infants)
Bulging of fontanelle
Arching of back
Positive Brudzinski/Kernig sign
Signs of shock
Focal neurological signs
Altered GCS
Papilloedema (rare)
199
Q

What investigations would you perform in meningitis?

A

Bloods: FBC, coagulation screen, U&Es, LFTs, CRP, glucose, blood gas and blood cultures.
Urine, throat swab for MC&S.
LP for CSF unless contraindicated.
Serum antigen test or PCR on blood or CSF.

200
Q

What is the management for meningitis?

A

Suspected cases of meningococcal meningitis in the community should receive IV/IM benzylpenicillin.
Suspected cases in hospital should receive 80mg/kg/OD ceftriaxone or 50mg/kg/TDS of cefotaxime.
Beyond the neonatal period, dexamethasone administered with Abx reduces the risk of long term complications e.g. deafness

201
Q

What is the prophylaxis for meningitis?

A

Rifampicin is given to all household contacts for meningococcal meningitis and haemophilus influenzae to eradicate nasopharyngeal carriage.

202
Q

What are the causes of encephalitis?

A

Direct invasion of the cerebrum by a neurotoxic virus e.g. HSV
Delayed brain swelling following a disordered neuroimmunological response to an antigen, usually a virus (e.g. following chickenpox)
A slow virus infection e.g. HIV or SSPE following measles

203
Q

What is the typical history associated with encephalitis?

A 
Fever
Headache
Photophobia
Lethargy
Poor feeding/vomiting 
Irritability 
Hypotonia/"floppiness"
Drowsiness
Loss of consciousness 
Seizures 
Altered mental state
204
Q

What are the examination findings in encephalitis?

A 
Fever
Neck stiffness (not always present in infants)
Bulging of fontanelle
Arching of back
Positive Brudzinski/Kernig sign
Signs of shock
Jaundice
GI bleeding
Purpura
Focal neurological signs
Altered GCS
205
Q

What investigations would you perform for encephalitis?

A

Bloods: FBC and films, U&Es, LFTs, ESR, CRP, glucose, gas and cultures.
LP for CSF MC&S
Later on, EEG, CT/MRI

206
Q

What is the management for encephalitis?

A

High dose IV aciclovir for at least 21 days if 3m-11y
3m-11d: 500mg/m2 every 8 hours
12-17y: 10mg/mg every 8 hours for at least 14 days

207
Q

What is the definition of a febrile seizure?

A

A febrile seizure is a seizure accompanied by a fever in the absence of intracranial infection due to bacterial meningitis or viral encephalitis. They occur in 3% of children.

208
Q

How are febrile seizures classified?

A

Simple - a generalised tonic-clonic seizure lasting <15m which does not recur within 24 hours or within the same febrile illness.
Complex - 1 or more of: focal features at the onset or during seizure, duration >15m, recurrence within the same febrile illness.
Febrile status epilepticus - >30m

209
Q

What is the aetiology of febrile seizures?

A 
Viral infections 
Otitis media
Tonsillitis 
Gastroenteritis
Post-immunisation 
RULE OUT: meningitis and septicaemia, UTI, LRTI, cerebral malaria (if the history suggests it)
210
Q

What is important information in the history of a febrile seizure?

A

Account of the seizure e.g. duration, postictal after?
Symptoms of a febrile illness
Past/FHx of febrile seizures or epilepsy

211
Q

What examinations should you do in a child presenting with a febrile seizure?

A 
Obs
GCS
Rash
Fontanelle
Rule of meningism 
Look for a focus of infection
212
Q

What investigations should you perform for a febrile seizure?

A

Bloods: FBC, U&Es, CRP, ESR, coagulation, glucose and culture.
Urine for MC&S
Consider an LP

213
Q

What is the management for a febrile seizure?

A

Reassure parents and give information and advice. If there is a history of prolonged seizures (e.g. >5m), rescue therapy with rectal diazepam or buccal midazolam can be used.

214
Q

What is epilepsy?

A

Epilepsy is a chronic neurological disorder characterised by recurrent, unprovoked seizures, consisting of transient signs and/or symptoms associated with abnormal, excessive or synchronous neuronal activity in the brain.

215
Q

What are the causes of childhood epilepsy?

A

Most epilepsy is idiopathic.
West syndrome
Lennox-Gasaut syndrome
Childhood absence epilepsy
Benign epilepsy with centrotemporal spikes (BECTS)
Early onset benign childhood occipital epilepsy
Juvenile myoclonic epilepsy

216
Q

What are the different types of childhood epileptic seizure?

A

Generalised: absence, myoclonic, tonic, tonic-clonic, atonic
Focal: frontal-motor phenomena; temporal - auditory, olfactory or taste phenomena; occipital - visual phenomena (positive or negative); parietal lobe - contralateral altered sensation

217
Q

How is childhood epilepsy usually diagnosed?

A

Usually based on a detailed history from the child and eyewitnesses

218
Q

What investigations would you perform for childhood epilepsy?

A

EEG
Structural imaging e.g. CT or MRI
Functional imaging e.g. PET and SPECT scans

219
Q

What is the management of childhood epilepsy?

A

Tonic-clonic: valproate, carbamazepine
Absence: valproate, ethosuximide
Myoclonic: valproate
Focal: carbamazepine, valproate, lamotrigine

220
Q

What are the core symptoms of ADHD?

A

Inattention, hyperactivity and impulsiveness.
Symptoms should be at developmentally inappropriate levels, be present across time and situations for at least 6 months and start before age 7.

221
Q

What are the risk factors for ADHD?

A

Low birth weight
Babies born to mothers who used drugs, alcohol or tobacco during pregnancy
Following head injury
Some genetic and metabolic disorders

222
Q

What are the clinical features of ADHD?

A

Inattention: careless with detail, fails to sustain attention, appears not to listen, fails to finish tasks, poor self organisation, loses things, forgetful
Hyperactivity: fidgets with hands or feet, leads their seat in class, runs/climbs about
Impulsiveness: talks excessively, blurts out answers, can’t wait their turn, interrupts others, intrudes on others

223
Q

What assessment does a child with ADHD need?

A

Interview the family and child
Observe the child (preferably in >1 setting)
Collateral information from school
Rating scales
Screen for comorbidity, physical examination

224
Q

What is the management of ADHD?

A

Psychoeducation, behavioural interventions, school intervention, treat comorbidity
Medication:
Methylphenidate - CNS stimulant (immediate release or modified release)
Atomoxetine - a non-stimulant NE reuptake inhibitor, taken OD
Dexamfetamine - CNS stimulant

225
Q

What is autism spectrum disorder?

A

The spectrum ranges from clear-cut autism to subtle variants, to traits found in the normal possiblation and includes autism, Asperger’s syndrome and pervasive developmental disorder - NOS

226
Q

What are the clinical features of an autism spectrum disorder?

A

Difficulties with social relationships: few or no sustained relationships, persistent aloofness or awkward interaction with peers, unusually egocentric with little concern for others or awareness of social rules. Problems with communication e.g. awkward or odd posture or body language
Absorbing and narrow interests: obsessively pursued and unusually circumscribed interests.

227
Q

What assessment should a child have for autism spectrum disorder?

A

History of problems including eliciting difficulties and level of distress and impairment in all aspects of life, comorbidity, cognitive ability, impact on parents/carers and sources of support. Obtain information from as many sources as possible.
Referral for specialist assessment e.g. SALT, OT, PT and educational psychology.
Observation of the child.
Consider the use of diagnostic tools.
Medical investigations as appropriate.

228
Q

What is the management for an autism spectrum disorder?

A 
Information and support for parents
Liaison with educational services 
Parenting programmes specific to ASD
Adaptation of the child's environment, activities and routines 
Communication interventions
229
Q

What is the aetiology of hypothyroidism in children?

A

Congenital: maldescent of the thyroid and athyrosis, dyshormonogenesis, iodine deficiency, hypothyroidism due to TSH deficiency - usually associated with panhypopituitarism
Acquired: autoimmune thyroiditis

230
Q

What are the clinical features of hypothyroidism in children?

A

Congenital: usually asymptomatic and picked up on screening, failure to thrive, feeding problems, prolonged jaundice, constipation, pale cold mottled dry skin, coarse facies, large tongue, hoarse cry, goitre (occasionally), umbilical hernia, delayed development
Acquired: females > males, short stature/growth failure, cold intolerance, dry skin, cold peripheries, bradycardia, thin dry hair, pale puffy eyes and loss of eyebrows, goitre, slow relaxing reflexes, constipation, delayed puberty, obesity, SUFE, deterioration in school work, learning difficulties.

231
Q

What investigations should you perform for hypothyroidism in children?

A

TFTs, thyroid antibodies
Thyroid USS
Radionucleotide scan

232
Q

What is the management of hypothyroidism in children?

A

Congenital: levothyroxine 10-15 micrograms/kg/day
Acquired: 25-200 micrograms/day

233
Q

What are the different types of precocious puberty?

A

True/central: premature activation of the hypothalamic-pituitary-gonadal axis
Pseudo/false: from excess sex steroids

234
Q

What is the aetiology of precocious puberty?

A

Central: idiopathic (familial/non-familial), intracranial tumour, other CNS lesions e.g. hydrocephalus, cranial irritation
Pseudo: Gonadal - McCune-Albright syndrome, ovarian tumours, testicular tumours, familial testitoxicosis, congenital adrenal hyperplasia, adrenal tumour, iatrogenic, HCG-secreting tumours e.g. CNS

235
Q

What examinations need to be done in precocious puberty?

A

Puberty/Tanner staging
Measure height, weight and head circumference
Review previous growth records if available
Measure parents heights
Look for skin lesions e.g. cafe-au-lait marks
Abdominal/testicular masses
Neurological examination

236
Q

What investigations should be performed for precocious puberty?

A 
plasma LH, FSH and sex hormone levels, 17-OH progesterone levels, DHEAS and androstenedione levels
Urine sample for steroid profile
Abdo and pelvic USS
MRI brain 
Bone age x-ray 
GnRH test
237
Q

What is the management of precocious puberty?

A

Central: long acting GnRH analogue

Treatment of cause

238
Q

What is congenital adrenal hyperplasia?

A

It describes a group of autosomal recessive disorders of cortisol biosynthesis. 21-alpha hydroxylase deficiency is the cause of approx 95% of cases and is characterised by cortisol deficiency +/- aldosterone deficiency and androgen excess

239
Q

What is the typical presentation in congenital adrenal hyperplasia?

A

Virilisation of the external genitalia in female infants with clitoral hypertrophy and variable fusion of the labia. In the infant male, the penis may be enlarged and the scrotum pigmented.
A salt-losing crisis in 80% of males who are salt losers - present at 1-3 weeks of age with vomiting, weight loss, floppiness and circulatory collapse.
Tall stature in the 20% of male non-salt losers.
Precocious puberty

240
Q

What investigations should you perform in congenital adrenal hyperplasia?

A

Bloods: Low Na, high K+, metabolic acidosis, hypoglycaemia, U&Es, glucose, blood gas, serum 17-hydroxyprogesterone
Pelvic USS
Karyotyping

241
Q

What is the management of congenital adrenal hyperplasia?

A

Immediate in salt-losing males: IV NaCl, dextrose and hydrocortisone
Lifelong glucocorticoids
Mineralocorticoids e.g. fludrocortisone if there’s salt loss and possible NaCl replacement therapy
Urogenital surgery for virilisation

242
Q

What causes iron-deficiency anaemia in children?

A

Inadequate intake of iron e.g. too much cows milk
Malabsorption
Iron

243
Q

What type of anaemia is iron-deficiency anaemia?

A

Microcytic, hypochromic

244
Q

What are the clinical features of iron-deficiency anaemia in children?

A

Most infants and children are asymptomatic until Hb <6-7g/dL.
Tire easily, young infants feed more slowly than usual, pallor and pallor the conjunctivae, tongue or palmar creases.
“Pica” - the inappropriate eating of non-food materials e.g. soil, chalk, gravel or foam rubber

245
Q

What investigations would you perform for iron-deficiency anaemia in children?

A

FBC and iron studies - monocytic hypochromic anaemia, decreased MCV and MCH, low serum ferritin
Other causes of microcytic anaemia: beta-thalassaemia trait, alpha-thalassaemia trait, anaemia of chronic disease

246
Q

What is the management of iron-deficiency anaemia in children?

A

Dietary advice and supplementation with oral iron - Sytron or Niferex

247
Q

What dietary advice should you give for iron-deficiency anaemia in children?

A

High in iron: red meat, liver, kidney, oily fish - pilchards, sardines
Average iron: pulses, beans, peas, fortified food
Avoid in excess in toddlers: cows milk, tea, high fibre foods

248
Q

What is the aetiology of ITP?

A

Destruction of circulating platelets by antiplatelet IgG autoantibodies. It most commonly occurs following a viral infection or occasionally following an immunisation.

249
Q

What are the clinical features of ITP?

A

Most children are aged 2-10y.
Onset is within 1-2w after a viral infection.
History is of petechiae, purpura and/or superficial bruising lasting days to weeks. Up to 25% present with epistaxis; GI bleeds and haematuria are rare.
Older girls may have menorrhagia. ICH is rare

250
Q

What investigations would you do for ITP?

A

FBC: platelets<10x10^9/L
Blood film
Bone marrow aspirate - only required if there are atypical features or the child is going to be treated with steroids (they mask ALL)

251
Q

What is the management of ITP?

A

In 80% of children, the disease is acute, benign and self-limiting. Usually remits spontaneously within 6-8 weeks. Most children can be managed at home.
Trauma prevention advice - avoid contact sports
Avoid aspirin and NSAIDs
Educate about when to seek medical assistance
Medical: prednisolone, IVIG, IV anti-D Ig (in Rh+ve children)

252
Q

What are the risk factors for leukaemia?

A 
Males>females
Down's syndrome 
Fanconi's anaemia
Ataxia telangiectasia 
Bloom's syndrome 
Significant exposure to ionising radiation 
Maternal AXR whilst pregnent
253
Q

What are the symptoms of leukaemia?

A 
General malaise
Fatigue
Lethargy
Prolonged or recurrent episodes of fever
Irritability
Growth restriction/FTT
SOB
Reduced exercise tolerance
Dizziness and palpitations
Epistaxis
Bleeding gums
Easy bruising 
Bone/joint pain
Troublesome constipation 
Prolonged cough
Headaches 
Nausea and vomiting 
Repeated/severe childhood infections
254
Q

What are the signs of leukaemia?

A 
Pallor secondary to anaemia 
Petechiae 
Purpura
Signs of severe infection 
Lymphadenopathy
Hepatosplenomegaly 
Expiratory wheeze 
Cranial nerve lesions or CNS pathology 
Testicular enlargement
255
Q

What investigations would you do for leukaemia?

A

FBC (not always abnormal)
Blood film
Bone marrow aspiration and biopsy
Imaging to determine the extent of the disease
Immunophenotyping and cytogenetic analysis
LP if CNS infiltration is suspected

256
Q

What is the management for leukaemia?

A 
Support for the family as well as the child 
Chemotherapy:
-induction 
-consolidation and CNS protection 
-interim maintenance 
-delayed intensification 
-continuing maintenance
Treat girls for 2y and boys for 3y
257
Q

What are some characteristic features of trisomy 21?

A

Craniofacial: round face, flat nasal bridge, upslanted palpebral fissures, epicanthic folds, brushfield spots in the iris, small mouth and protruding tongues, small ears, flat occiput and third fontanelle
Other: short neck, single palmar crease, incurved 5th finger, wide sandal gap between toes, hypotonia, congenital heart defects, duodenal atresia, Hirschsprung’s disease

258
Q

What are some medical problems patients with trisomy 21 may suffer with later in life?

A

Delayed motor milestones, moderate-severe learning difficulties, small stature, increased susceptibility to infections, hearing impairment, visual impairment - cataracts and squints, increased risk of leukaemia, solid tumours, low thyroid, coeliac disease, epilepsy, Alzheimer’s disease

259
Q

What are some features of trisomy 13 - Patau’s?

A 
Structural defect of the brain - holoprosencephaly 
Small for gestational age 
Microcephaly 
Microphthalmia 
Cleft lip/palate
Congenital heart defect 
Renal anomalies 
Polydactyly
260
Q

What are some features of trisomy 18 - Edwards?

A 
Small for gestational age
Congenital heart disease - usually VSD +/- valve dysplasia
Short sternum
Over-riding fingers
"Rocker bottom feet"
261
Q

What is the presentation in Klinefelter Syndrome?

A

Can be variable and often delayed. Children may present with delayed speech or learning difficulties, unusually rapid growth in mid-childhood or truncal obesity. Adults may present with hypogonadism (hypergonadotrophic) or subfertility. There may also be failure of sexual maturation.

262
Q

What is the genetic abnormality in Klinefelter Syndrome?

A

47XXY

263
Q

What are the clinical features in Klinefelter Syndrome?

A

Infertility, small firm testes, decreased facial and pubic hair, loss of libido, impotence, tall and slender with long legs, narrow shoulders and wide hips, gynaecomastia (or history of), history of undescended testes. Learning disability, delayed speech development, behavioural problems and psychosocial disturbances.

264
Q

How is Klinefelter Syndrome diagnosed?

A

Decreased serum testosterone
Increased FSH and LH (FSH>LH)
Karyotyping

265
Q

How is Klinefelter Syndrome managed?

A

Testosterone replacement - should begin as they enter puberty

266
Q

What is the genetic abnormality in Turner Syndrome?

A

45X

267
Q

What are the clinical features of Turner Syndrome?

A

Lymphaoedema of the hands and feet in neonates (may persist), spoon-shaped nails, short stature - cardinal feature, neck webbing or thick neck, wide carrying angle, widely spread nipples, congenital heart defects (particularly coarctation of the aorta), delayed puberty, ovarian dysgenesis (primary amenorrhoea and infertility), hypothyroidism, renal abnormalities (e.g. horseshoe kidney), pigmented moles, recurrent otitis media, low posterior hairline, normal intellectual function in most

268
Q

How is Turner Syndrome managed?

A

Growth hormone therapy
Oestrogen replacement for the development of secondary sexual characteristics at the time of puberty - infertility persists

269
Q

What is the aetiology of osteogenesis imperfecta?

A

It is an inherited condition causing increased fragility of bone due to a mutation in type I collagen gene that predisposes to fracture formation

270
Q

What are the different types of osteogenesis imperfecta?

A 
Type I - mildest form, AD
Type II - lethal form, AD
Type III - severely progressive, AR
Type IV - moderately severe, AD
Types 5-7 don't have the same genetic mutations as types 1-4
271
Q

What are the clinical features of osteogenesis imperfecta?

A

Bones: low birth weight/length for gestational age, short stature, 50% scoliosis
Joints: ligamentous laxity resulting in hyperextensible joint
Wormian bones on skull XR
Frequent fractures, blue sclera, cardiac involvement e.g. aortic regurgitation, mitral valve prolapse and aortic root widening

272
Q

What investigations would you do for osteogenesis imperfecta?

A

Prenatal USS may detect severe forms in the foetus, molecular genetic testing.
Biochemistry: raised alk phos (may be normal)
Skin biopsy - assess collagen in cultured fibroblasts
Bone biopsy - increased Haversian canal and osteocyte lacunae diameters, increased cell numbers

273
Q

What is the management of osteogenesis imperfecta?

A

No curative treatment - aim to prevent and manage fractures
Prevention: oral calcium supplements, bisphosphonates, synthetic calcitonin
Surgical: intramedullary rods to prevent bowing of long bones, especially fractures in children >2y. Corrective surgery for scoliosis deformity >50 degrees

274
Q

What is the aetiology of septic arthritis?

A

<12m: S. aureus, group B strep, Gram negative bacilli, Candida albicans
1-5y: S. aureus, Hib, Group A strep, S. pneumoniae, Kingella kingae, N. gonorrhoeae (child abuse)
5-12y: S. aureus, Group A strep
12-18y: S. aureus, N. gonorrhoeae (sexually active)

275
Q

What are the symptoms of septic arthritis?

A

Infants characteristically do not appeal ill, 50% do not have fever.
In older children, acute onset, hot warm and swollen joint, limping, inability to weight bear, systemic symptoms of infection.

276
Q

What are the signs of septic arthritis?

A

Decreased range of movement
Pain on passive motion
Joint effusion may be detectable in peripheral joints

277
Q

What investigations would you perform in septic arthritis?

A

Bloods: FBC, ESR, CRP, cultures, Lyme titres if exposed
XR of joint - normal initially, subluxation, joint space narrowing and erosive changes
Joint aspiration +/- USS for MC&S
MRI or CT
Bone scan
LP if caused by Hib (increased incidence of meningitis)

278
Q

What is the management of septic arthritis?

A

Medical: IV Abx AFTER aspirate is taken, for up to 3 w (until inflammatory markers settle) followed by oral Abx for a total of 4-6w.
Surgical: early referral for irrigation and debridement of affected joint if needed
Splintage: improves pain and allows inflammation to settle
Physio: avoid joint stiffness

279
Q

What is the pathogenesis involved in osteomyelitis?

A

Infection of the metaphysis of long bones, most common sites are the distal femur and proximal tibia. It’s usually spread via the haematogenous route from a primary site of entry but can also occur after open fractures or penetrating wounds.
S. aureus is the most common pathogenesis

280
Q

What are the different types of osteomyelitis?

A

Acute
Subacute - 2-3w duration
Chronic

281
Q

What is the typical presentation of osteomyelitis?

A

Neonates characteristically do not appear ill and may not have a fever.
Pain, limp, refusal to walk/weight bear, fever, malaise, flu-like symptoms.
Immobile limb (pseudoparesis).
Over the infected site there is swelling and tenderness, may be erythematous and warm.
Effusion in an adjacent joint.

282
Q

What is the DDx for osteomyelitis?

A

JIA
Lyme/post-streptococcal arthritis
Acute leukaemia
Neoplasm

283
Q

What investigations would you perform for osteomyelitis?

A 
Bloods: FBC, ESR, CRP, cultures
XR (may be normal in the early stages)
USS-guided aspiration for MC&amp;S
MRI
Bone scan
(biopsy)
284
Q

What is the management for osteomyelitis?

A

Medical: IV Abx for a minimum of 2w and then oral Abx for 4w
Surgical: drainage or debridement if there’s frank pus on aspiration or an abscess/collection

285
Q

What are the complications of osteomyelitis?

A

Systemic: septicaemia
Local: pathological fracture, sequestration, growth disturbance

286
Q

What are the risk factors for developmental dysplasia of the hip?

A 
Family History
Female > Male (5:1)
Left>right
Racial predilection
Breech presentation
287
Q

What is the aetiology of developmental dysplasia of the hip?

A 
Capsular laxity (increased type III collagen, maternal oestrogens)
Decreased uterine volume - breech presentation, first born, oligohydramnios
288
Q

What is the disease progression in develpmental dysplasia of the hip?

A

Capsular laxity and shallow acetabulum Instability/subluxation/dislocation
Muscle contracture
Progressive acetabular dysplasia with a fibro-fatty substance filling the acetabulum
Hypoplastic femoral head

289
Q

What tests are done to screen for developmental dysplasia of the hip?

A

2 tests done on NIPE
Ortolani’s test - try to reduce (already dislocated hips)
Barlow’s test - is the hip dislocatable?

290
Q

What is the presentation in developmental dysplasia of the hip?

A

Infant: asymmetric gluteal folds, limited abduction, leg length discrepancy
Older child: may walk with a limp, positive Trendelenburg test

291
Q

What investigations should be performed if developmental dysplasia of the hip is suspected?

A

<6m: USS of the hip

>6m: AP pelvis radiograph

292
Q

What is the management of developmental dysplasia of the hip?

A

<6m: Pavlik Harness

Referral to orthopaedic surgeon

293
Q

What is Perthes disease?

A

Idiopathic avascular necrosis of the capital femoral epiphysis of the femoral head due to interruption of the blood supply followed by revascularisation and reossification

294
Q

What are the risk factors for Perthes disease?

A 
Boys > girls (5:1)
Age 4-10y
Low birth weight 
Low socio-economic status 
Short stature 
Passive smoking
295
Q

What are the symptoms of Perthes disease?

A

Mild/intermittent anterior thigh/groin/referred knee pain with a limp
Classical “painless limp”

296
Q

What are the signs of Perthes disease?

A 
Proximal thigh atrophy 
Mild short stature
Limp/Trendelenburg/antalgic gait 
Effusion from synovitis 
Groin/thigh tenderness
Decreased hip ROM (abduction and internal rotation) with muscle spasm
297
Q

What investigations would you perform for Perthes disease?

A

XR of both hips including frog views, repeat may be needed if clinical symptoms persist
Bone scan
MRI
Roll test - external and internal rotation invoke guarding/spasm

298
Q

What is the management of Perthes disease?

A

If identified early, bed rest and traction
In more severe disease/late presentations, the femoral head needs to be covered by the acetabulum - achieved by maintaining the hip in abduction with plaster or calipers or by performing femoral or pelvis osteotomy

299
Q

What is SUFE?

A

The displacement of the epiphysis of the femoral head postero-inferiorly requiring prompt treatment in order to prevent avascular necrosis

300
Q

What are the risk factors for SUFE?

A 
Local trauma 
Obesity 
Neglected septic arthritis
Hypothyroidism 
Hypopituitarism 
Growth hormone deficiency 
Vit D deficiency 
Previous radiation of the pelvis 
Chemotherapy
301
Q

What is the presentation of SUFE?

A

Usually occurs in 10-15 year olds.
Pain in the hip, groin, medial thigh or knee during walking.
Limping.
Pain in accentuated by running, jumping or pivoting.
Onset can be acute or insidious.
May be able to weight bear but it’s painful.
Flexion of the hip also causes external rotation, may be leg shortening.

302
Q

What is the DDx for SUFE?

A 
Acute hip #
Perthes disease
Transient synovitis 
Osteomyelitis 
Septic arthritis 
DDH
303
Q

What investigations should you perform for SUFE?

A

XR of the hip (AP and frog leg views) - widening and irregularity of the plate of the femoral epiphysis

304
Q

What is the management of SUFE?

A

Avoid moving or rotating the leg, patient shouldn’t walk.
Analgesia and immediate orthopaedic referral.
Surgical pinning of the hip is usually required and should be done quickly.

305
Q

What is the pathophysiology of transient synovitis?

A

Non-specific inflammation of the synovial lining of the hip, often 1-2w after an URTI

306
Q

What are the symptoms of transient synovitis?

A

Acute limp
Non-weight bearing
Mild fever or no fever
Child is still able to walk with difficulty
Child looks well and is comfortable at rest

307
Q

What are the signs of transient synovitis?

A

Systemically well
Limited internal rotation and pain on movement
Reduction in ROM
Often able to weight bear with encouragement

308
Q

What is the important DDx of transient synovitis?

A

IMPORTANT TO RULE OUT SEPTIC ARTHRITIS

309
Q

What investigations should you perform in transient synovitis?

A

XR (AP, lateral and frog views) - usually normal, may show some joint space widening
Bloods: FBC, U&E, CRP, ESR - ESR may be slightly raised or normal
MRI or USS if unsure of diagnosis
If there is any suspicion of septic arthritis, joint aspiration and blood cultures MUST be done

310
Q

What is the management of transient synovitis?

A

Bed rest, analgesia and mobilisation once the pain settles

311
Q

What are the different types of JIA?

A

Oligoarticular JIA (=4 joints)
Polyarticular JIA RF negative (>4 joints)
Polyarticular JIA RF positive (>4 joints)
Systemic onset JIA
Juvenile psoriatic arthritis
Enthesitis-related arthritis
Undifferentiated arthritis

312
Q

What are the symptoms of JIA?

A 
Persistent joint swelling >6w duration 
Stiffness after periods of rest e.g. long car rides, morning joint stiffness
High fever
Salmon coloured pink rash 
Lethargy 
Reduced physical activity
Poor appetite
Deterioration in behaviour/mood
313
Q

What are the signs of JIA?

A

Bloods: FBC, ESR/CRP (increased), ANA, RF, HLA B27, serology if post-infectious arthritis is suspected
Imaging e.g. XR (normal in early JIA)
USS (joint effusion, synovial hypertrophy and erosions)
MRI (bony changes, joint damage)

314
Q

What is the management of JIA?

A

MDT approach - paediatric rheumatology, ophthalmology, ortho, PT, OT and rehab specialists
NSAIDs, steroids, DMARDs e.g. methotrexate, sulfasalazine, leflunomide. Etanercept. Tocilizumab
Surgery: joint replacement or synovectomy

315
Q

What are the complications of JIA?

A

Anterior uveitis
Flexion contractures of the joints
Growth failure
Constitutional problems e.g. anaemia of chronic disease, delayed puberty, osteoporosis
Psychosocial, behavioural and educational difficulties

316
Q

What is the aetiology of respiratory distress syndrome?

A

Deficiency of surfactant which lowers surface tension, surfactant is a mixture of phospholipids and proteins excreted by type II pneumocytes of the alveolar epithelium.
Surfactant deficiency leads to alveolar collapse and inadequate gas exchange.

317
Q

What are the risk factors for respiratory distress syndrome?

A 
Premature delivery 
Infants delivered via LSCS without maternal labour 
Hypothermia 
Perinatal asphyxia 
Maternal diabetes 
FHx
Intrapartum asphyxia
Pulmonary infection 
Pulmonary haemorrhage
Meconium aspiration 
O2 toxicity and barotrauma 
Diaphragmatic hernia
318
Q

What is the presentation of respiratory distress syndrome?

A 
Tachypnoea >60 breaths/min
Laboured breathing with chest wall recession (particularly sternal and subcostal) and nasal flaring 
Expiratory grunting 
Diminished breath sounds 
Cyanosis
319
Q

What investigations should you do in respiratory distress syndrome?

A

Blood gases
Pulse oximetry
CXR - ground glass appearance of the lungs and air bronchograms
Bloods: FBC, U&E and creatinine, LFTs, glucose, cultures to exclude sepsis
Echo

320
Q

What is the management of respiratory distress syndrome?

A

Raised ambient O2 supplemented with CPAP or I&V if needed
Surfactant replacement therapy
Supportive therapy

Phase 3A (9 decks)

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