Paeds - General Flashcards
Bronchiolitis aetiology
- Most common viral LRTI in infants
- RSV Infection
respiratory syncytial virus
RF for severe bronchiolitis and protective factors
- Age (<3m)
- Prematurity (<32w)
- Haemodynamically significant CHD
- CF
- Congenita/acquired lung disease (incl. bronchopulmonary dysplasia)
- Immunodeficiency
- Neuromuscular disorders
- FHx of atopic disease
Protective
• BF
• Parental avoidance of smoking
Bronchiolitis ddx
Unusual in children <1 years of age:
Pneumonia
• High fever (>39) and/or
• Persistent focal crackles
Viral induced wheeze/ early onset asthma
• Persistent wheeze without crackles or
• Recurrent episodic wheeze or
• Personal family hx of atopy
Bronchiolitis epidemiology
- Children <2 years
- Most common in the first year of life
- Peaks between 3-6 months
Croup aetiology
• Usually caused by a virus
o Typically parainfluenza virus types 1 or 3
o Parainfluenza 1 is the most common type
o Parainfluenza virus epidemics tend to occur every other year
• Bacterial croup is less common
o Mycoplasma pneumoniae
o Corynebacterium diphtheriae
• Symptoms due to upper-airway obstruction due to generalised inflammation of the airways as a result of viral infection
Croup epidemiology
- 6months-6 years
- Peak incidence – 2nd year
- Peak hospital admissions – September-December
- M>F
Croup ddx + symptoms/signs
• Bacterial tracheitis o Fever >39 o Sudden onset stridor o Respiratory distress o Following viral-like respiratory illness from which the person appears to be recovering but then becomes acutely worse o Partial/no response to adrenaline
• Epiglottitis o Sudden onset high fever >39 o Dysphagia o Drooling o Anxiety o Non-barking cough o Preferred posture – sitting upright with head extended o Rare o No response to adrenaline
• Upper airway foreign body
o Sudden onset dyspnoea
o Stridor
• Retropharyngeal/peritonsillar abscess o Fever o Dysphagia o Drooling o Stridor (occasionally) o Dyspnoea o Tachypnoea o Neck stiffness o Unilateral cervical adenopathy o More gradual onset than with coup
• Angioneurotic oedema
o Acute swelling of the upper airway – may cause dyspnoea and stridor
o Swelling of the face, tongue, pharynx
• Allergic reaction
o Rapid onset dysphagia, stridor, possible cutaneous manifestations (urticarial rash)
o Personal or FHx of prior episodes, atopy
Croup vs epiglottitis vs tracheitis
Incidence Age Aetiology Speed of onset Fever Cough Voice Position Neck XR AP Neck XR lateral Response to adrenaline
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Whooping cough clinical dx
• Had an acute cough for >=14d without another apparent cause + has >=1 of the following
o Inspiratory whoop
o Post-tussive vomiting
o Paroxysmal cough
o Undiagnosed apnoeic attacks in young infants
Whooping cough causative organism
incubation period
duration that the person is infectious for
when do symptoms resolve
• Bacterium Bordetella pertussis
o Incubation period – 7 days
o Most infectious in catarrhal phase
o Person is infectious for 3 weeks after the onset of symptoms if no antibiotics are given
• Considered to be infectious from onset of symptoms until
o 48h of appropriate abx treatment
o 21 days from onset of symptoms if appropriate abx therapy has not been completed
• Spread by aerosol droplets
o Catarrhal phase
1-2 weeks
Sx of URTIs – nasal discharge, conjunctivitis, malaise, sore throat, low-grade fever, dry, unproductive cough
Pertussis is rarely diagnosed during this stage unless there has been contact with a person who is known to be infected
Whooping cough complications
• Serious: o Apnoea o Pneumonia (usually caused by 2o bacterial infection) o Seizures o Encephalopathy (rare in adults)
• Less serious
o Otitis media in children (caused by 2o bacterial infection)
o Unilateral hearing loss (v rarely reported)
• Increased intra-thoracic pressure + intra-abdominal pressure due to violent and/or prolonged coughing
o Pneumothorax
o Umbilical + inguinal hernias
o Rectal prolapse
o Rib fracture
o Herniation of lumbar intervertebral discs
o Urinary incontinence
o Subconjunctival or scleral haemorrhage
o Facial and truncal petechiae
• Severe dehydration +/or malnutrition
Whooping cough prognosis
• Considered to be infectious from onset of symptoms until
o 48h of appropriate abx treatment
o 21 days from onset of symptoms if appropriate abx therapy has not been completed
- People who have not previously contracted whooping cough + are not vaccinated- whooping cough causes a protracted cough which may last >3 months (100-day cough) despite antibiotic treatment
- Immunised people - shorter lived, milder symptoms, isolated persistent cough
o A previously infected person can become re-infected with pertussis but subsequent infections are usually less severe
o Vaccination does not always prevent infection but it usually attenuates the disease
• Mortality rate for children <6 months - 3.5%
o Apnoea associated with paroxysms may cause sudden death
- Mortality rate in the general population - 0.03%
- Future URTI may produce whooping for a while afterwards
Whooping cough ddx
• Other causes of URTI and LRTI
o Adenoviral infection – fever, sore throat, conjunctivitis
o M. pneumonae – fever, headache, systemic symptoms
o Chlamydophila pneumoniae – pharyngitis, bronchitis, atypical pneumonia, mainly elderly and debilitated patients
o B. parapertussis – similar but milder illness. Immunity to B. pertussis does not confer immunity to this different organism
- Asthma
- COPD
- Post-infectious cough
- Upper airway cough syndrome
- GORD
- Lung malignancy
Pneumonia causative organisms in
neonates
infants-preschool children
older children
• Neonates
o Organisms from the female genital tract: GBS, E. coli, and gram-negative bacilli, chlamydia trachomatis
• Infants-preschool children:
o Viral (most common)
Parainfluenza, influenza, adenovirus and RSV
RSV can be particularly dangerous to ex-preterm infants and infants with underling chronic lung disease (CLD) of prematurity.
o Bacterial
Streptococcus pneumonia (90% of bacterial pneumonia)
Staphylococcus aureus is uncommon but causes severe infection
• Older children-adolescents
o As above
o Also atypical organism such as Mycoplasma pneumonia and chlamydia pneumoinae
o M. pneumoniae occurs in outbreaks approx. every 4 years + it is more common in school-aged children
o Legionella pneumophilia
o Chlamydophila pneumoniae
o Coxiella burnetiid
• TB should be considered at any age
Pneumonia in children
causative organisms in
aspiration pneumonia
non-immunised patients
immunocompromised patients
• Aspiration pneumonia
o Enteric gram-negative bacteria +/- Strep. Pneumonia, staph aureus
• Non-immunised
o Haemophilus influenza, Bordetella pertussis, measles
• Immunocompromised (inherited or acquired):
o Viral: CMV, VZV, HSV, measles and adenoviruses
o Bacterial: Pneumocystis carinii, TB
Pneumonia in children prognosis
• Most resolve within 1-3 weeks
Pneumonia in children complications
• Bacterial invasion of lung tissue o Pneumonic consolidation o Septicaemia o Empyema o Lung abscess (esp. S aureus) o Pleural effusion o Pleural effusion o ARDS, ARF
• Previous lung disease/immunocompromised
o Respiratory failure
o Hypoxia
o Death
Mesenteric adenitis prognosis
- Symptoms usually improve within a few days
- Symptoms always clear up completely within 2 weeks
- Bacterial infection needs to be treated
Mesenteric adenitis ddx
- Mesenteric ischaemia
- Chronic abdominal pain
- IBD
- UTI
- Intussusception
- Appendicitis
- Ectopic pregnancy
- Ovarian cyst rupture
- Ovarian abscess
- Endometriosis
- Ovarian torsion
- PID
- Testicular torsion
- Epididymitis
- SLE
- Malignancy
- HIV
- Zoonotic infections
- Infectious mononucleosis
- TB
Mesenteric adenitis cause
• Non-specific inflammation of the mesenteric lymph nodes which provokes a mild peritoneal reaction + stimulates painful peristalsis in the terminal ileum
• Need to exclude biliary atresia in any baby with
• Need to exclude biliary atresia in any baby with
o Jaundice associated with pale stools
o Jaundice beyond 14 days of age
o If direct or conjugated bilirubin is >17.1 micromoles/L (1mg/dl)
Biliary atresia cause
idiopathic
unknown aetiology
Biliary atresia complications
• Growth failure – most common indication for liver transplantation
• Cholangitis
o Most serious complication – requires prompt identification + treatment with IV abx for 10/7 min + up to 6 w.
o Can cause rapid progression of liver disease + death
o Fever, jaundice, acholic stools, irritability
o Typically occurs in the first 2 years of life
• Fat soluble vitamin deficiency - ADEK
o Vitamin D malabsorption – bone fractures, rickets, osteomalacia, osteopenia
- Portal hypertension
- GI bleed – from varices
- Ascites
Biliary atresia prognosis
• Fatal without surgery
• Leading indication for paediatric liver transplantation
o 70% of children with biliary atresia will undergo liver transplantation, 50% of them by the age of 2 years
• Even with appropriate, timely surgical intervention it is often an unrelenting inflammatory process
o Hepatoportoenterostomy – transplant-free survival for at least 2 years in about 50% of children
o If total bilirubin is <34.2micromoles/L (<2mg/L) at 3 months post-HPE, then the chance of being transplant free at 2 years of age is 84%
Biliary atresia ddx
- Extrahepatic biliary obstruction (e.g. choledochal cyst, spontaneous perforation of CBD, vile duct stricture/tumour, neonatal sclerosing cholangitis)
- Hepatic viral infections (e.g. CMV, enterovirus, HSV, adenovirus, HBV, HIV, rubella, parvovirus B18, EBV)
- Alpha-1 antitrypsin deficiency (Fhx of lung disease at an early age)
- CF
- Hypothyroidism – predominantly unconjugated hyperbilirubinemia
- Galactosaemia
- Mitochondrial disorders
- Peroxisomal disorders – craniofacial + neurological abnormalities in association with jaundice
- Toxic causes – drugs, TPN, endotoxin from gram -ve bacteria
- Sepsis
• Ischaemia-reperfusion injury
o Typically occurs after an infant has been profoundly ill
- Inpissated bile – In CF or after significant haemolysis
- CHD
Atresia vs stenosis
o Atresia – a complete blockage (obstruction) or lack of continuity of the bowel
o Stenosis – a partial obstruction that results in narrowing or stricture of the bowel
Intestinal atresia is associated with which conditions
Duodenal atresia
30% DS
30% CHD
Jejunoileal atresia
10% CF
Intestinal atresia complications and prognosis
Complications • Polyhydramnios o risk of PTL • Mechanical bowel problems • Feeding + absorption difficulties – refer to paediatric dietician
Prognosis
• Excellent long-term outcomes
Intestinal atresia ddx
- Malrotation with midgut volvulus (proximal obstruction)
- Meconium ileus (distal ileal obstruction)
- Long-segment Hirschsprung disease (associated with colonic ateresia)
List the 3 types of cerebral palsy
• Spastic o 70% o UMN lesion o Tight/stiff muscles o Hypertonia o Jerky movement o Scissor gait o Toe walk
• Athetoid (dyskinetic) o Injury to basal ganglia o Involuntary movements o Dystonia – random, slow, uncontrolled, movements in limbs/trunk o Chorea – random, dance like movements
• Ataxic o Damage to cerebellum o Shaky or uncoordinated o Clumsy o Poor balance
What kind of disorder is cerebral palsy?
- Non-progressive neurodevelopmental disorders
* Group of permanent movement and posture disorders that limit activity
Primary causes of cerebral palsy
o Prematurity
o Multiple gestation
o Maternal infection e.g. chorioamnionitis
AN RF for cerebral palsy
o Multiple gestation
o Maternal illness – can affect the developing brain
Chorioamnionitis and/or fever
Maternal respiratory tract or genito-urinary infection treated in hospital
TORCH
Maternal thyroid disease
o Maternal teratogen exposure
Indirect RF
Exposure to teratogens (alcohol, cigarettes, antenatal XR, warfarin) – rf for prematurity + LBW
o Maternal thrombotic disorders Inc. Factor V Leiden mutations – can lead to neonatal emboli from placental thrombosis
o Fetal brain malformation
o Fetal genetic and metabolic disorders
o Placental abruption
perinatal RF for cerebral palsy
o Prematurity
o LBW
o Respiratory distress
o Fetal birth asphyxia – birth trauma, placental abruption, uterine rupture, prolonged labour, instrumental delivery
o Multiple births
o Intraventricular haemorrhage
o Neonatal encephalopathy
o Neonatal complications
Severe hyperbilirubinemia – damage to basal ganglia – dyskinesia
Severe periventricular haemorrhage
Neonatal sepsis (particularly if BW <1.5kg)
• Peri-natal hypoxic-ischaemic injury
postnatal RF for cerebral palsy
• Postnatal (10%)
o Head injuries prior to 3 years
o Meningitis
• Familial metabolic/genetic disorder
cerebral palsy complications
- Feeding + swallowing difficulties – 50% - can lead to poor growth
- Speech impairment – 30%
- Communication difficulties – 50%
- Sialorrhoea (drooling) – may be affected by positioning/medication/history/reflux/dental issues
• Low bone mineral density (osteopenia, osteoporosis)– esp. if
o Non-ambulant (GMFCS level IV or V)
o Vit D deficiency
o Eating/drinking/swallowing difficulties or concerns about nutritional status
o LQ for age (<2nd centile)
o Hx of low-impact #
o Use of anti-convulsant medication
• Secondary MSK problems
• Intellectual disability
o LD (IQ <70) – around 50% of children w CP
o Severe LD (IQ <50) – around 25% of children w CP
- Pain
- Sleep disturbance
• Behavioural + mental health problems
o Behavioural difficulties (problems w behaviour/ peer relationships/ attention/ concentration/ hyperactivity/conduct) – 30%
- Epilepsy – 30%
- Neurogenic bladder – Urinary incontinence
- Chronic constipation – 60%
- GORD
- Variable sensory/proprioceptive loss
• Additional disturbances of sensation/perception/cognition/communication/behaviour
o Visual impairment – around 50% of children w CP (strabismus, refractive errors, problems of eye function, retinopathy of prematurity, impaired cerebral visual information processing, visual field defects)
o Hearing impairment – around 10% of children w CP
cerebral palsy prognosis
- Permanent condition
- Underlying brain lesion is non-progressive
- Functional and neurological manifestations evolve over time
• Walking
o The more severe the child’s physical/functional/cognitive impairment, the greater the possibility of difficulties with walking
o If a child can
Sit by 2– likely that they will be able to walk unaided by 6
Cannot sit but can roll at 2 – possibility that they may be able to walk unaided by 6
Cannot sit or roll at 2 – unlikely to be able to walk unaided
• Speech development
o 50% have some difficulty with elements of communication
o 33% have specific difficulties w speech + language
o 10% need augmentative + alternative communication (signs, symbols, speech generating devices)
• Increased prevalence of
o Mental health + psychological problems incl. depression, anxiety, conduct disorders
o Neurodevelopmental disorders incl. ASD, ADHD
• Reduced QOL
• Reduced Life expectancy
o The more severe the child’s physical/functional/cognitive impairment, the greater the likelihood of reduced life expectancy
cerebral palsy ddx
• Spinal muscular atrophy
o No spasticity
o Pt may develop contractures
o Floppy at birth + exhibits progressive weakness
• Muscular dystrophy/myopathy o No spasticity o Pt may develop contractures o Normal development until 3 years of age followed by progressive loss of function + muscle weakness o Positive FHx
• Spinal stenosis/tethered cord
o Non-spastic
o Progressively worsening neurological function
• Familial/primary dystonia
o Onset of muscular deformity after several years of normal development
o Sustained periods of muscle contraction + dystonia
o No development of contractures
o Abrupt + violent movements
o Positive FHx
• Familial (hereditary) spastic paraparesis
o FHx
o Progressive disease
• Myelodysplasia
o Associated spinal defect
o Lack of sensation below specific spinal segment
o Usually non-spastic
• Brain tumour
o Acute presentation – headache, ICP (early morning vomiting), seizures, focal neurological deficit following initial normal development
Atopic eczema - severity
- Clear
- Mild
- Moderate
- Severe
• Clear
o Normal skin
o No evidence of active atopic eczema
o Emollients should still be used
• Mild
o Areas of dry skin
o Infrequent itching (+/- small areas of redness)
• Moderate
o Areas of dry skin
o Frequent itching
o Redness (+/- excoriation + localised skin thickening)
• Severe
o Widespread areas of dry skin
o Incessant itching
o Redness (+/- excoriation, extensive skin thickening, bleeding, oozing, cracking, alteration of pigmentation)
Atopic eczema dx
• Should be diagnosed when a child has an itchy skin condition + >3 of
o Visible flexural dermatitis involving skin creases (bends of elbows, behind knees) or visible dermatitis on cheeks +/or extensor areas in children <18m
o PHx of flexural dermatitis or dermatitis on cheeks +/or extensor areas in children <18m
o PHx of dry skin in the last 12 months
o PHx of asthma or allergic rhinitis (or hx of atopic disease in first-degree relative of children <4)
o Onset of signs + symptoms <2 y/o (should not be used in children aged <4 years)
Atopic eczema trigger factors
• Trigger factors o Irritants e.g. soaps, detergents (incl. shampoos, bubble baths, shower gels, washing-up liquids) o Skin infections o Contact allergens o Food allergens o Inhalant allergens
• Unclear trigger factors that should be avoided where possible
o Stress
o Humidity
o Extremes of temperature
Eczema prognosis
• Condition improves with time but not all children will grow out of atopic eczema + it may get worse in teenage/adult life
o Many cases of atopic eczema clear or improve during childhood – others, can persist into adulthood
- Flares may occur as frequently as 2-3 per month
- Some children who have atopic eczema will go on to develop asthma or allergic rhinitis = atopic march
Eczema ddx
- Psoriasis — less itchy, well-circumscribed, reddish, flat-topped plaques with silvery scales; typically symmetrical.
- Allergic contact dermatitis — eczematous rash, at any site related to a topical allergen, in a person of any age. Can be both an alternative diagnosis and a trigger factor of atopic eczema
- Seborrhoeic dermatitis — red, sharply marginated lesions with greasy scales; usually confined to areas with sebaceous gland activity (for example ears, beard area, eyebrows, scalp, and nasolabial folds)
- Fungal infection — annular patch or plaque with slightly raised, sometimes scaly, border, and central clearing
- Scabies or other infestations — should be suspected when there is recent onset of an itchy rash in a family
• Ddx for eczema herpeticum
o Impetigo
o Hand, foot, mouth disease
Pathophysiology of DMD + BMD
• Boys do not produce functional dystrophin protein (DMD), abnormal/partially functional dystrophin (BDM) weakness of skeletal, respiratory, heart muscles shortened life span
X-linked
DMD + BMD complications
• Respiratory failure
o Most common cause of morbidity + mortality
- Loss of mobility
- Weight loss/malnutrition
- Sexual dysfunction
• Cardiac complications
o Cardiomyopathy + CHF
o Atrial + ventricular arrhythmias
o Dilated cardiomyopathy is common
• Smooth muscle affected
o GI symptoms e.g. gastric dilation, pseudo-obstruction
• Hypersomnolence + morning headaches – due to nocturnal hypoventilation
• Learning difficulty
o Non progressive
o May affect verbal ability more than other performance
• Late complications of daily CS use o Osteoporosis o Vertebral compression fractures o Failure of boys to enter puberty o Obesity o HTN
• Anaesthetic complications
o Myoglobinuria
o Rhabdomyolysis
o Malignant hyeperthermia
DMD prognosis
DMD
• Progressive muscle weakness
o Hand muscles spared until late on
o Extraocular muscle function preserved
• 10-40% of patients survive to age 40 in centres that use non-invasive means of respiratory support
• Most patients
o Lose the ability to walk by 12 years of age
o Require ventilatory support by 25 years of age
• Average life expectancy – 27 years – respiratory or cardiac complications
BMD prognosis
BMD
• Reduced life-expectancy (middle to old age)
• 50% deaths due to cardiomyopathy
DMD, BMD ddx
• BMD
• Limb-girdle muscular dystrophies
o CK levels usually lower
• Emery-Dreifuss muscular dystrophy
• Polymyositis
o 4-7 y/o
o Muscle contractures + weakness can become as severe as in DMD
o Muscle weakness usually occurs in the proximal muscles – esp. those of the shoulder + pelvic girdle
o Muscle biopsy – inflammation, mononuclear invasion of non- necrotic muscle, CD8+ cytotoxic/suppressor T cells, macrophages, absence of perifascicular atrophy of dermatomyositis
• Static encephalopathies (cerebral palsy)
o Children affected asymmetrically
o Clinical dx
CNS involvement
Spasticity (not seen in neuromuscular diseases)
Cognitive/motor/sensory involvement
o DMD – hypotonic, symmetrically affected
• Neurological causes of muscle weakness – spinal cord lesions, spinal muscular atrophy, MND, MS
When might you want to investigate a child for DMD
Ix DMD in any boy who
• Is not walking by 18m
• Has delayed motor milestones or global developmental delay
What is West syndrome?
Infantile spasms • Epilepsy syndrome • Diagnostic triad – west syndrome o Flexor, extensor, mixed spasms o Developmental plateau or regression o Hypsarrhythmia or modified hypsarrhythmia on EEG
• Age of onset = 1 month – 1 year
o Median age – 3-5 months
Infantile spasms/West syndrome RF
• Brain malformation
o Particularly lissencephaly
• Neurocutaneous syndrome
o Tuberous sclerosis
- AN or perinatal vascular event – stroke, preterm intraventricular haemorrhage, hypoxic-ischaemic encephalopathy, birth hypoxia, traumatic brain injury
- Intrauterine or perinatal infections – HSV, CMV, toxoplasmosis, HIV
- Inherited metabolic disorders
- Genetic disorders e.g. T2, genes CDKL5, ARX
- Brain tumours – astrocytoma, ganglioglioma
Infantile spasms/West syndrome complications
• Adverse effects of hormonal therapy
o HTN, hyperglycaemia, increased susceptibility to infections (particularly VZV), increased appetite, wight gain, irritability, gastric irritation, altered sleep-wake pattern
• SE of vigabatrin
o Lethargy, irritability, sleeping + feeding difficulties, constipation, hypotonia, vigabatrin-associated visual defects (irreversible)
Infantile spasms/West syndrome prognosis
• 5-30% mortality
• Poor prognosis
Loss of skills
Learning disabilities
Continuing epilepsy
• Survivors
o 80-90% developmental delay – may precede or follow onset of seizures
o 50-70% other seizure types
Lennox-Gastaut syndrome – predominance of tonic + atonic seizures + atypical absence seizures, psychomotor retardation
Focal seizures with impaired awareness
- Reduced life expectancy – depends on aetiology of infantile spasms
- Rapid identification + treatment of the spasms improves prognosis
• Poor prognostic factors o Ealy age of onset (<3m) o Structural brain abnormalities o Presence of pre-existing neurological impairment or developmental delay, Seizure types occurring before onset of spasms o Co-occurrence of other seizure types o Lack of response to ACTH therapy
• Favourable prognostic factors
o No apparent aetiology
o Age at onset > 4 m
o Absence of atypical spasms and focal seizures
o Absence of asymmetrical electroencephalogram abnormalities
o Prompt treatment and an early and sustained response to treatment
o Normal psychomotor development before the onset of spasms
Infantile spasms/West syndrome ddx
• Dravet syndrome – Severe myoclonic epilepsy of infancy
o Prolonged, repeated febrile + afebrile generalised or unilateral convulsive seizures
o Interictal myoclonus and ataxia
o Nocturnal generalised tonic-clonic seizure
o Mild to severe intellectual disability
• GORD
o Reflux may mimic seizures
o Normal EEG + development
• Benign…
o …Familial infantile seizures
Normal psychomotor development
Seizures resolve within the first year of life
Normal inter-ictal EEG, diffuse discharge with onset in central occipital region in ictal EEG
o … Neonatal sleep myoclonus Non-epileptic condition Myoclonic jerks during sleep stop abruptly when child is aroused Resolves by 1 year of life Normal EEG
o … myoclonus of early infancy
Similar symptoms
Normal EEG
Normal development – no mental and psychomotor involvement
Gastroenteritis causative organisms
• Most cases are due to an enteric virus
o Rotavirus – most common cause of viral gastroenteritis in children
o Norovirus – prevalence during colder months
o Adenoviruses
• Some are caused by bacterial or protozoal infections
o Bacteria
Campylobacter jejuni, Cambylobacter coli
E. coli
Salmonellosis – red white meats, raw eggs, milk, dairy products
Shigella
Yersinia
o Parasites
Cryptosporidosis
Amoebiasis
Giardia
• Faeco-oral, foodborne, environmental, airborne transmission
• Food poisoning
o Primarily caused by enterotoxins produced by the microorganism
Gastroenteritis causative RF
- Contact with infected person
- Contaminated water or food – Exposure to known source of enteric infection
- Recent travel abroad
Timeframe for D+V to improve in gastroenteritis
o D – 5-7d, in most it stops within 2w
o V – 1-2d, in most it stops within 3d
In which cases is gastroenteritis considered a notifiable disease
Notifiable disease - notify the local health protection team by completing a notification form if gastroenteritis caused by
• Food poisoning (such as suspected Bacillus cereus, Campylobacter spp., Clostridium perfringens, Cryptosporidium spp., Entamoeba histolytica, verocytotoxigenic Escherichia coli [including E. coli O157:H7], Salmonella spp., Giardia lamblia, and Yersinia pestis), including suspected clusters or outbreaks.
• Haemolytic uraemic syndrome.
• Infectious bloody diarrhoea, such as Shigella spp.
• Enteric fever (typhoid or paratyphoid fever).
• Cholera.
When is oral rehydration therapy CI in gastroenteritis?
CI to use of oral rehydration therapy* • Protracted vomiting • Impaired consciousness • Paralytic ileus • Monosaccharide malabsorption
Children at increased risk of dehydration
• At increased risk of dehydration o <1y, particularly <6m o LBW o >5 D stools in the previous 24h o >2 V in the previous 24h
o Children who have not been offered/have not been able to tolerate supplementary fluids before presentation
o Infants who have stopped BF during the illness
o Children with signs of malnutrition
Complications of gastroenteritis
• Volume depletion/ dehydration
o Severe diarrhoea can cause dehydration which may be life-threatening
• Electrolyte imbalance
o Dehydration can be hyponatraemia, isonatraemic, hypernatraemia
o Hyponatraemia – if plain water, carbonated drinks, fruit juices are used solely in the oral rehydration process or hypotonic saline used for IV rehydration
o Hypokalaemia – loss of K+ in urine, increase in aldosterone in an attempt to conserve sodium – most common occurrence in severe dehydration
- Metabolic acidosis – fecal loss of bicarbonate, renal excretion of H+
- Hypoglycaemia
- HUS – haemolytic uraemic syndrome in children with Shiga-toxin producing E. coli infection
• NG
o Trauma to nose, oesophagus, stomach
o Aspiration
• IV o Interstitial infiltration o Fluid at cannula site o Pain o Bleeding o Phlebitis o Seizures
Gastroenteritis prognosis
• Illness usually resolves without treatment within days
o D – 5-7d, in most it stops within 2w
o V – 1-2d, in most it stops within 3d
• Most children can be safely managed at home
What is DDH
+ epidemiology
- Spectrum of conditions affecting the proximal femur and acetabulum
- Range from acetabular immaturity with a stable hip, to neonatal hip instability, established hip dysplasia +/- later subluxation and frank hip dislocation
- True DDH = femoral head has a persistently abnormal anatomical relationship with the pelvic acetabulum abnormal bony development that can ultimately result in premature arthritis + significant disability
- Transient dysplasia = acetabular immaturity – relationship stabilises and normalises over a period of weeks to months
- F>M
- Affects 1-3% of newborns
- 20% of cases bilateral
- L hip dislocated more often than R
DDH aetiology
- The hip is a ball and socket joint that is not fully developed at birth
- Much of the development of the hip joint occurs in utero and within the first several months of life
- Normal development of the hip joint requires appropriate alignment and contact between the ball of the femoral head and the socket of the acetabulum
- In persistent DDH, the anatomical relationship between the femoral head and the acetabulum is incorrect, leading to abnormal development
DDH RF
• Breech presentation • FHx • Restricted intrauterine space o First pregnancy o Oligohydramnios o Macrosomia o Multiple gestation
- Feet first
- Female
- Fluid (oligohydramnios)
- Fatty (>5kgs)
- First born with Family history
- Foot deformity (calcaneovalgus deformity)
DDH complications
• Nerve palsy
o Might occur secondary to suboptimal positioning within the brace, forced abduction and/or excessive flexion
o Most common nerve palsy seen with the use of a Pavlik harness – femoral nerve palsy – loss of active knee extension – nerve palsies usually recover
• Pavlik harness disease
o Extended use of Pavlik harness (>3-4w) – worsening of posterolateral acetabular erosion and dysplasia
• AVN
o Also called proximal femoral growth disturbance – caused by excessive pressure on the femoral head + compression of the extrinsic blood supply of the femoral epiphysis
o Can occur with any of the treatment methods used for DDH
o AVN with Pavlik harness – from 0-7%
o AVN with surgery - 5-15%
o Poor prognostic factor
• Surgical complication
o Re-dislocation
o Stiffness
o Blood loss
• Subsequent surgery
o Surgery needed by almost 6% of infants treated with abduction splinting
o Residual acetabular dysplasia after closed/open treatment of hip instability
• Long-term complications
o Premature degenerative joint disease
o Low back pain
• Untreated unilateral hip dislocation
o Limb length inequality – may cause gait alteration and low back and/or knee pain
o Knee valgus
o Back pain
DDH prognosis
• Factors associated with poorer prognosis
o Age at time of intervention – remodelling potential is more reliable in younger children + within the first 12-18 months after reduction is achieved – remodelling potential remains but is likely reduced during the subsequent years of growth
o High dislocation
o Residual subluxation
o Evidence of AVN
o Osteonecrosis
o Re-dislocation
- Important cause of childhood disability
- Accounts for up to 9% of all primary hip replacements – 29% of those in people <60
DDH ddx
- Acetabular immaturity – repeat US over several weeks to differentiate from DDH
- Residual effects of septic arthritis – acute presentation w symptoms of septic arthritis
- Fracture of femoral neck – uncommon in infancy
What is Perthes disease
• Disease of the femoral head, comprising of necrosis, collapse, repair, re-modelling
Osteochondritis
- The essential lesion is loss of blood supply (avascular necrosis) of the nucleus of the proximal femoral epiphysis abnormal growth of the epiphysis eventual remodelling of regenerated bone
- Single or multiple vascular events causing femoral head infarct, followed by re-vascularisation
- Can be arterial or venous
Perthes disease epidemiology
• Presents in the first decade of life
• Age 4-8
o Mean age = 7 y/o
o Can affect children between 2-12 years of age
• Bilateral in 15%
• M>F
• Disease appears very uncommon in black people
Perthes disease complications
• Stiffness and loss of rotation
o Maintaining hip mobility throughout the disease process helps prevent this complication
• Osteoarthritis
o Severe deformity of the femoral head – early arthritis
• Limb length inequality
o Damage to the blood supply of the proximal femoral epiphysis may lead to its premature closure
• Hinged abduction – occurs when an enlarged femoral head is pushed laterally + it impinges on the acetabular rim when the hip is abducted
• Residual deformities
o Coxa magna – broadening of the head + neck of the femur
o Coxa plana – osteochondritis of the femoral head
o Coxa breva – structural shortening of the neck of femur
Perthes disease prognosis
- Most children have good outcomes
- In later life, it can lead to a painful + poorly functioning hip
- At least 50% of involved hips do well without treatment
- > 50% of patients with Perthes’ disease will develop signs of osteoarthritis between their 4th and 5th decades – might require hip replacement
o For patients who are <6 outcome is good regardless of treatment
• Prognostic factors
o Age
o Limitation of movement
o Radiologically visible involvement of the femoral head
o Lateralisation of the femoral head in the acetabulum (subluxation)
o Lateral epiphyseal calcification
o Metaphyseal cyst formation
Perthe’s disease ddx
• Most likely dx for an acute limp in a child aged 3-10 years
o Transient synovitis of the hip
o Fracture or soft tissue injury (incl. stress fracture or NAI)
• Bilateral Perthes’ disease
o Hypothyroidism
o Multiple epiphyseal dysplasia (Meyer’s disease)
Bilateral epiphyseal dysplasia with both hips at the same stage with the same degree of involvement
Bilateral Perthes’ disease is more likely to be sequential than simultaneous
o Spondyloepiphyseal dysplasia tarda
Bilateral Perthes’ disease with spine involvement
Spine involvement = platyspondyly – flattened vertebral body shape with reduced distance between end plates
o Sickle cell disease
Acute abdominal pain
Systemic symptoms
• Unilateral Perthes’ disease o Septic arthritis o Sickle cell disease o Spondyloepiphyseal dysplasia tarda o Gaucher’s disease o Eosinophilic granuloma o Transient synovitis • Juvenile idiopathic arthritis o Rh factor +ve o Systemic symptoms
What is Osgood-Schlatter’s disease (OSD)
• Overuse syndrome, affects the knees
• Usually unilateral
o Affects both knees in 20-30% of people
• Inflammation of the patellar ligament where it inserts on the tibial tuberosity
o When the tuberosity hasn’t ossified yet (adolescents) not strong enough to resist traction of the ligament excessive strain of patellar ligament inflammation of the ligament = traction apophysitis
o Excessive traction ossification centre can crack callus formation
Healing + regrowth of the avulsed fragments – minimal to marked firm enlargement of the tibial tubercle
• Results in traction apophysitis of the tibial tubercle
o Apophysis = bony prominence that serves as a site for tendon attachment
o Apophysitis = inflammation or stress injury to the areas on or around growth plates in children and adolescents
Osgood-Schlatter’s disease (OSD) RF
• Athletic participation in sports that involve repeated knee flexion + forced extension – running, jumping, squatting, deep knee bending, track, rugby, basketball, baseball, football
• History of OSD in contralateral knee
• Biomechanical RF
o Quadriceps muscle tightness
o Reduced flexibility of hamstring muscles
Osgood-Schlatter’s disease (OSD) epidemiology
- Typically affects young athletes during their adolescent growth spurt
- Boys 12-15 y/o, girls 8-12 y/o
- Affects about 1 in 10 adolescents + up to 1 in 5 adolescents who participate in sport involving high impact activities e.g. running, jumping
- M>F
Osgood-Schlatter’s disease (OSD) prognosis
- Symptoms typically settle over weeks or months
- Occasionally may persist for 1-2 years before resolving completely in 90% of people
- Resolution when patients reach skeletal maturity – complete recovery expected when the tibial growth plate closes around 14-18 years of age
- Up to 60% may have longer-term pain + reduced function
- Up to 10% of patients may experience pain as adults
- Persistent Osgood-Schlatter disease has been associated with chronic pain, reduced ability to participate in sports, decreased lower body strength, lower QOL
- Excellent relief of symptoms can be expected after surgical treatment of refractory cases
Osgood-Schlatter’s disease (OSD) ddx
• Tumour
o Severe, persists at night or at rest, may be associated with bone or joint pain at other sites
o FLAWS
o Osteoid osteoma– pain at rest + with activity, night time pain
o Osteosarcoma – pain at rest + with activity, systemic symptoms
• Trauma
o Patellar stress fracture – pt may report a sensation of crack or pop with activities, variable ability to bear weight
o Fracture of the tibial tubercle – Acute, trauma, unable to actively extend knee, unable to bear weight
o Patellar tendonitis
o Infrapatellar bursitis – pain at or near patellar insertion, no tenderness over tibial tubercle
o Meniscal injuries
o Collateral and cruciate ligament injuries
• Infection
o Osteomyelitis – pain at rest + with activity, systemic symptoms
o Septic arthritis
• Inflammatory arthritis
o Juvenile idiopathic arthritis – affects knee joint alone or multiple joint – joint pain, swelling, morning stiffness
• Referred pain from the hip
o Slipped capital femoral epiphysis
o Transient synovitis
o Perthes disease
• Other causes
o Osteochondritis dissecans of the knee – anterior/anteromedial knee pain, intermittent knee swelling, mechanical symptoms, hx of minor trauma or atraumatic, absence of tenderness at the tibial tubercle
o Patellar dislocation or subluxation, patellofemoral pain syndrome, chondromalacia pattelae
What is hand foot and mout disease?
What is it caused by?
- Acute viral illness
- Also known as enteroviral vesicular stomatitis
Aetiology
• Coxsackie virus
o Coxsackie A16 is the most common cause
o Coxsackie A6 – atypical HMFD – more widespread + extensive skin disease, nail shedding and higher risk for adult infection
• Incubation period is 3-7 days
• Mucocutaneous lesions appear 1-2 days after the prodromal period – last approx. 7-10 days with spontaneous resolution
RF + transmission of hand foot and mouth disease
- Immunosuppression
- Children <10
- Family/school contacts with infection
• Spread by
o Direct contact with the nasal + throat secretions of an infected person
o Direct contact with fluid from the blisters
o Faeco-oral transmission
- Transmitted immediately before and during the acute stage of the illness
- Faecal shedding can persist for approx. 4-8 weeks after the onset of the illness
- Vertical spread from mother to fetus
Hand foot and mouth disease complications
• Dehydration – oral pain causing reduced fluid intake
• Secondary bacterial infection
o Patients with localised or diffuse spreading skin erythema and/or persistent fever should be evaluated for infection and considered for empirical oral abx coverage
• EV71 – life-threatening sequelae e.g. brainstem encephalitis, aseptic meningitis, acute flaccid paralysis (polio-like syndrome), autonomic dysregulation pulmonary oedema + haemorrhage, myocardial impairment, myocarditis – 10-26% mortality rates
o Encephalitis/encephalomyelitis – Acute or sub-acute onset of febrile illness, altered mental status, focal neurological abnormalities, seizures
o Admit to hospital for symptomatic + supportive treatment
• HFMD in pregnancy – miscarriage, stillbirth, congenital malformation, IUGR, hydrops fetalis
• HFMD acquired around the time of delivery – neonatal infection
o Mild + self-limiting
o Neonatal complications – severe hepatic/respiratory/neurological disease
Hand foot and mouth disease prognosis
- Infection typically resolves spontaneously within 10-14 days
- Spontaneous recovery with no complications – follow-up not usually required
• Coxsackie A16
o Mild, self-limiting
o Oral + skin lesions nearly always fully resolve without treatment in 7-10 days
• Coxsackie A6
o Self-limiting
o Initial acute phase followed by desquamation of the palms + soles for several weeks
o Onychomadesis within 3-8 weeks of symptom onset
• Enterovirus 71
o Higher incidence of neurological involvement (4%) (e.g. aseptic meningitis, encephalitis, encephalomyelitis)
o Pulmonary oedema, pulmonary haemorrhage, myocarditis, polio-like syndrome, death
o 8% mortality
Hand foot and mouth disease ddx
- Chickenpox – itchy rash, mainly limited to trunk + extremities, round vesicle on an erythematous base that evolves into a pustule
- Herpangina – coxsackie A viruses, high fever/malaise/headache/oral lesions/cervical lymphadenopathy – no associated exanthema (rash)
- Herpes stomatitis
• Herpes simplex
o High fever, acute gingivitis, widespread oral ulceration
o Small vesicles, oval, erythematous base, grouped together
o No rash on the palms/soles – young children who suck their toes may have lesions on 1 or 2 digits
• Aphthous ulcers – can become herpetiform + lead to stomatitis
o Well child
o No rash
o No fever/malaise
- Viral pharyngitis
- Kawasaki disease
- Pompholyx eczema
- Bechet’s disease
- Pemphigus vulgaris or oral bullous pemphigoid
Chickenpox
definition
RF
Epidemiology
- Acute disease caused by VZV
- Incubation period – 1-3 weeks
- Infectious from 1-2 days before the rash appears until the vesicles are dry or have crusted over – usually 5 days after the onset of the rash
RF • Hx of recent exposure to chickenpox • Significant exposure o Timing of exposure in relation to the rash onset in the index case from 48h before onset of rash to crusting lesions (usually 5 days after) o Closeness + duration of contact Maternal/neonatal contact Continuous home contact Contact in the same room for >15 mins or contact on large open wards F2F contact e.g. having a conversation • Age 1-9 • Unvaccinated • Occupational exposure
Epidemiology • Very infectious – up to 90% of susceptible contacts develop the disease • Transmission o Personal contact o Droplet spread • Highest incidence before 10 years of age • >90% of people >15 years are immune • Peak incidence – from March to May
Chickenpox complications
Neonates
• At increased risk of disseminated or haemorrhagic varicella
• If mother becomes infected 1-4 weeks before delivery
o Up to 50% of babies will be infected
o 25% will develop clinical varicella of the newborn, even though they have passively acquired maternal antibody
• If mother becomes infected at 20-27 weeks of pregnancy
o Baby may develop shingles of infancy or early childhood due to reactivation of the primary in utero infection
Children
• Skin bacterial superinfection e.g. impetigo, furuncles, cellulitis, erysipelas, necrotizing fasciitis, scarring
o May manifest as high grade pyrexia (often after initial improvement)
o Erythema
o Tenderness surrounding original chickenpox lesions
• Neurological complications e.g. Reye’s syndrome, acute cerebellar ataxia, encephalitis, meningoencephalitis, polyradiculitis, myelitis
• Congenital varicella syndrome (If varicella in pregnant women during the 1st or 2nd trimester
o Cutaneous skin lesions
o Limb hypoplasia or paresis
o Microcephaly
o Ophthalmic lesions
• Rare – myocarditis, glomerulonephritis, appendicitis, pancreatitis, Henoch-Schonlein purpura, orchitis, arthritis, optic neuritis, iritis, keratitis
Adults
• More serious complications, more likely to be admitted
• Pneumonia, hepatitis, encephalitis
• Shingles
Chickenpox prognosis
- Self-limiting disease
- No F/U necessary
- In 30% of people, VZV reactivates later in life as shingles or herpes zoster
Chickenpox ddx
• Vesicular viral rashes
o HS – not usually disseminated
o Herpes zoster (shingles) – usually unilateral + localised to dermatomes
o Hand, foot and mouth disease
• Other infections o Impetigo o Scabies o Syphilis o Meningococcaemia (can be confused with haemorrhagic varicella) o Toxic shock syndrome
• Skin disorders o Guttate psoriasis o Drug eruption o Insect bites o Popular urticaria o Erythema multiforme o SJS/TEN Lesions can look like targets initially but eventually become flaccid blisters Erythroderma, pain in involved areas Blisters can become confluent + are associated with a positive Nikolsky’s sign – epidermal layers easily sloughs off when pressure is applied to the affected area Hx of exposure to a medication associated with the condition o Henoch-Schoenlein purpura o Dermatitis herpetiformis
Mumps causative agent+ incubation period + most infectious period + mode of transmission
- Paramyxovirus
- Incubation period = 16-18d
- Most infectious from around 1-2 days before onset of symptoms to about 9 days afterwards
- Spread by respiratory droplets, fomites or saliva
Mumps complications
- Rare
- Occur more frequently among adults than children
• Epidydymo-orchitis
o Unilateral mumps epididymo-orchitis can significantly but only transiently diminish the sperm count, mobility and morphology
o Bilateral mumps epididymo orchitis causes infertility in 30-87% of affected men
o Presentation – acute painful swelling of the testicle, systemic symptoms (high fever 39-40, chills, headache, vomiting)
o O/E – affected testicle is usually enlarged (up to 4x the normal size), warm, tender + the scrotum may be reddened in appearance
o Usually unilateral, tends to occur about 1 week after symptoms of parotitis
- Oophoritis – N, V, lower abdominal pain
- Encephalitis
• Aseptic/Viral meningitis
o Occurs about 4 days after parotitis
o Usually benign – fever, headache, vomiting, neck stiffness, lethargy
o Peaks after 2 days
o Resolves over the course of about 1 week
- Transient hearing loss
- Myocardial complications
• Pancreatitis
o Upper abdominal discomfort
o Mild and transient
Mumps prognosis
- Self-limiting disease
- Resolves within 1-2 weeks
- Most people recover without any long-term complication
- Nearly all people develop life-long immunity to mumps after one episode of infection
Mumps ddx
• Infectious causes that may present with parotitis
o Viral infections – EBV, parainfluenza, adenovirus, influenza type A, coxsackievirus, parvovirus B19, lymphocytic choriomeningitis virus, HIV
o Acute suppurative parotitis – acute bacterial infection (Staph aureus, atypical bacteria (e.g. TB))
• Non-infectious causes of parotitis
o Parotid duct obstruction – salivary stones, cysts, tumours
o Prescription drugs – thiazide diuretics, phenothiazines, thiouracil, iodide contrast media
o Metabolic disorders – DM, cirrhosis, uraemia
o AI diseases – sarcoidosis, Sjogren’s syndrome, Wegener’s granulomatosis
Measles causative agent+ incubation period + most infectious period + mode of transmission
- Morbillivirus of the paramyxovirus family
- Incubation period – 10 days
- Person is infectious form when the symptoms first appear (around 4 days before the rash appears) to 4 days after the onset of the rash
• Airborne infection – spread by
o Droplets from coughing + sneezing
o Close personal contact
o Direct contact with nasal or throat secretions
Measles complications
• Susceptibility to opportunistic infection
• Secondary infections of the respiratory tract
o Otitis media (7-9% of cases)
o Pneumonitis
o Tracheobronchitis
o Pneumonia (1-6% of cases)
• CNS complications
o Convulsions
o Encephalitis
o Blindness
o SSE (subacute sclerosing panencephalitis) – rare, degenerative disease of the CNS involving seizures + decline in motor, cognitive, behavioural function
o Up to 16x more common in children who contract measles at a very young age (<1y)
o Occurs a median 7y after exposure to the virus – may occur as late as 3 decades afterwards – is invariably fatal
• Diarrhoea
• Complications more severe in
o Children <5/Adults/pregnancy/immunocompromised/ chronically ill or malnourished children/ infants
o Infants – more likely to require hospitalisation than older children + are at higher risk of pneumonia, otitis media, SSPE, mortality due to measles
Measles prognosis
- Most people with measles make a full recovery with symptomatic management after around 7 days of sx
- Death from measles is rare in developed countries
- Once infected, the person develops lifelong immunity
- Susceptibility to opportunistic infection may last months to years after the measles illness
Measles ddx
• Parvovirus B19 o Bright red rash on cheeks o Red/lacy rash on the rest of the body o No Koplik’s spots o Adults – arthralgia + arthritis
• Streptococcal infection (e.g. scarlet fever)
o Maculopapular rash
o Appears on the abdomen + spreads to the back + limbs 12-24h following symptom onset
o Sore throat – usually a prominent symptom
o Cough not generally a feature
o Strawberry tongue
• HHV6 (roseola infantum)
o Mild illness – children look well (those with measles typically look + behave as if they are unwell)
o May be asymptomatic
o Fever can last for 3-5 days after which a maculopapular rash appears (when clinical improvement has occurred)
o Rash starts from torso + spreads out
• Rubella
o Mild
o If fever is present, it rarely occurs after the first day of the rash
o Post auricular and sub-occipital lymphadenopathy
o Rash is maculopapular but not confluent
o Usually starts behind the ears + on the face + then spreads down the body (similar to measles)
o No Koplik’s spots
• Early meningococcal disease
o Maculopapular rash
o Becomes pruritic in later stages
o Does not fade when a glass is pressed against it
• Other causes of rash to consider that are associated with lymphadenopathy include Kawasaki disease + infectious mononucleosis
Immunisation schedule
vaccines for
8w
12w
16w
8w
DTaP/IPV/Hib/HepB – 6-in-1
MenB
Rotavirus
12w
DTaP/IPV/Hib/HepB – 6-in-1
Rotavirus
PCV
16w
DTaP/IPV/Hib/HepB – 6-in-1
MenB
o IPV = Salk – inactivated vaccine
Immunisation schedule
vaccines for
1y
3y + 4m
12-13y
14y
1y Hib/Men C PCV booster MMR Men B booster
3y + 4m
DTaP/IPV – 4-in-1
MMR (2nd dose, check first dose given)
12-13y
HPV
14y
Td/IPV – 3-in-1
MenACWY
o IPV = Salk – inactivated vaccine
Which children get the nasal spray influenza vaccine?
Annually from 2 years old
All primary school children (reception to year 6)
All year 7-year 11 children in secondary school
Aged 2-17 with long-term health conditions*
*if <9 + long-term health condition + never had a flu vaccine before 2 doses, 4 w apart
live vaccine
Which children can’t get the nasal spray influenza vaccine?
If between 6 months + 2 years + long term health condition
Flu vaccine injection
(nasal spray not licensed for <2)
Vaccination contraindications
Consider whether to avoid specific vaccinations in the following
• Acute febrile illness
• Individuals with a hx of a confirmed anaphylactic reaction to a previous dose of the vaccine / to a component of the vaccine
o Egg allergy Influenza Tick borne encephalitis Yellow fever Hep A Recent studies suggest that anaphylactic reactions to MMR are not associated with hypersensitivity to egg antigens all children with egg allergy should receive the MMR but can be given in hospital if egg allergic
• Do not give live vaccines
o Individuals with primary or acquired immunodeficiency– includes
Immunosuppression due to acute + chronic leukaemia + lymphoma
Severe immunosuppression due to HIV/AIDS – BCG CI in all HIV+ individuals
Cellular immune deficiencies (SCIDS, DiGeorge syndrome)
Being under follow up for a chronic lymphoproliferative disorder incl. haematological malignancies
Having received an allogenic/autologous SCT in the past 2m
o Individuals on current/recent immunosuppressive therapy
High dose CS (>40mg prednisolone per day) for >1w
Low dose CS (>20mg prednisolone per day) for >2w
If immunocompromised for short-term – delay vaccines
o Infants born to mothers who received immunosuppressive therapy during pregnancy – delay for 6 months
o Those in contact with an individual with immunodeficiency/recent immunosuppressive therapy
o Pregnant women
Vaccination for
babies born to HBV infected mothers TB RF (born in a high incidence country/ parent/grandparent born in a high incidence country)
babies born to HBV infected mothers
birth, 4w, 12w
Hep B vaccine
take blood for HBsAg at 12 months to exclude infection
TB RF (born in a high incidence country/ parent/grandparent born in a high incidence country)
at birth
BCG
Vaccination for
asplenic or splenic dysfunciton (incl. due to sickle cell + coeliac disease)
Chronic respiratory/heart/neurological conditions)
diabeted
CKD
chronic liver conditions
haemophilia
immunosuppression due to disease or treatment
complement disorders
asplenic or splenic dysfunciton (incl. due to sickle cell + coeliac disease)
Men ABCWY
Pneumococcal
Influenza
Chronic respiratory/heart/neurological conditions)
Pneumococcal
Influenza
diabetes
Pneumococcal
Influenza
CKD
Pneumococcal (stage 4, 5)
Influenza (stage 3, 4, 5)
Hepatitis B (stage 4, 5)
chronic liver conditions Pneumococcal Influenza Hep A Hep B
haemophilia
Hep A
Hep B
immunosuppression due to disease or treatment
Pneumococcal
Influenza
complement disorders
Men ABCWY
Pneumococcal
Influenza
Live vaccines currently available in the UK
Listen Very Silently My Rude BOY • Live influenza vaccine (Fluenz Tetra) • Varicella vaccine (Varilrix, Varilvax) • Shingles vaccine (Zostravax) • MMR (Priorix, MMRVaxPro) • Rotavirus vaccine (Rotarix) • BCG vaccine • Oral typhoid vaccine (Ty21a) • Yellow fever vaccine
Live vaccine menmonic
ROME is my best place to go yet
BOYs Love The CRIME
https://pbs.twimg.com/media/DKBeg2tW4AA3uxl.jpg
Oral polio vaccine = sabin = live
Killed vaccines mnemonic
- Rest in peace always
- Rabies
- Influenza
- Polio (salk) - IPV - this is the vaccine being used for childhood immunisations in the UK (injection)
- Hepatitis A, hepatitis B
https://pbs.twimg.com/media/C_oj9VKXoAAA_N2.jpg
When to dx CF
- Positive results + no symptoms (e.g. infant screening – blood spot immunoreactive trypsin test) followed by sweat + gene tests for confirmation or
- Clinical manifestations + sweat/gene test results confirming CF or
- Clinical manifestation alone
CF aetiology + RF
• ΔF 508 mutation
• Abnormalities in the CFTR (CF transmembrane conductance regulator)
o Cl- channel found in cells lining the lungs, intestines, pancreatic ducts, sweat glands, reproductive organs
o >2000 known disease-causing mutations that interrupt various stages of CFTR synthesis + function
• Abnormalities in salt + water transport across epithelial surfaces
• FHx
• Known carrier status of both parents
• Mutations in CFTR result in abnormal salt transport by epithelial cells, resulting in thick, sticky secretions.
• In the pancreas, this leads to blockage of exocrine ducts, early activation of pancreatic enzymes, and eventual autodestruction of the exocrine pancreas. Therefore, most patients require supplemental pancreatic enzymes.
• In the intestine, bulky stools can lead to intestinal blockage.
• In the respiratory system, the absence of CFTR function results in mucus retention, chronic infection, and inflammation that eventuate in the destruction of lung tissue
CF complications + prognosis
• GI complications o Underweight/malnutrition o Intussusception o Meconium ileus o Fat soluble vitamin deficiencies - ADEK o Distal intestinal obstruction syndrome o Chronic liver disease o CF related diabetes (uncommon in children <10y, prevalence with age and it affects up to 50% of adults) • Muscle pains, arthralgia • Male infertility caused by obstructive azoospermia (almost all males with CF are infertile) • Reduced female fertility • BMD (incl. osteoporosis) • Severe CF – delayed puberty • Pulmonary complications o Pneumonia o Upper airway complications – nasal polyps, sinusitis • USI • Survival to almost 40 years of age
CF ddx
- Primary ciliary dyskinesia – chronic purulent middle ear infections
- Primary immunodeficiency
- Asthma
- GORD
- Chronic aspiration
- Failure to thrive
- Coeliac disease
- Protein losing encephalopathy – loss of protein through the GIT – can be associated with Fontan’s procedure, lymphatic disorders, mucosal erosion
- If distal intestinal obstruction syndrome – constipation, appendicitis, intussusception, cholecystitis
Describe constipation
<3 complete stools per week (type 3 or 4)
What might be contributing to idiopathic constipation?
IIn idiopathic constipation you really need to spend time exploring the social hx of the family!
• Obvious precipitating factors coinciding with the start of symptoms: fissure change of diet infections timing of potty/toilet training moving house starting nursery/school fears and phobias major change in family taking medicines
Constipation ddx
- Hirschprung’s disease (i.e. congenital megacolon)
- Fissure
- Colitis
- Spinal or neuromuscular abnormalities
- Cerebral palsy
- Hypothyroidism
- Anal stenosis
- Imperforate anus with fistula
- Allergy or sensitivity to cow’s milk
- Coeliac disease
Constipation prognosis
- Symptoms become chronic in >1/3 of patients
- Constipation is a common reason for referral to secondary care
- There is suitable treatment for idiopathic constipation but it may take several months for the condition to be resolved
- Some children + young people may require laxative therapy for several years
- A minority may require ongoing laxative therapy
- Secondary behavioural problems are common
Pyloric stenosis - what is it and describe epidemiology
- Hypertrophy of the pyloric sphincter narrowing of the pyloric canal
- Most common cause of gastric outlet obstruction in the 2 to 12- week old age group
- Typical patient – 3-6 w old infant (usually male)
- May occur in older infants
Pyloric stenosis complications and protngosis
Pyloromyotomy
• Wound infection
• Gastric or duodenal mucosal perforation
• Incomplete myotomy
• Postoperative emesis
• Success of surgical treatment is near 100%
• Indirect hyperbilirubinemia – resolves with hydration
• Early recognition – metabolic abnormalities are less common
Pyloric stenosis ddx
- GORD
- Over-feeding – infants will not have difficulty gaining weight
- Malrotation – bilious emesis
- Acute infectious diarrhoea – pyloric stenosis usually presents with constipation
- Food allergy
- Duodenal/jejunoileal atresia – bilious emesis
- Pyloric atresia – non-bilious emesis
Define
GOR
GORD
Regurgitation
• GOR
o Effortless spitting up of 1 or 2 mouthfuls of stomach contents
o Passage of gastric contents into the oesophagus
o Normal – considered physiological in infants when symptoms are absent or not troublesome
o Not GORD
• GORD
o Presence of troublesome symptoms or complications arising from GOR
o Marked distress
o Feeding difficulties
o Faltering growth
• Regurgitation (also known as “posseting”)
o Voluntary + involuntary movement of part or all of the stomach contents up the oesophagus at least as far as the mouth + often emerging from the mouth
o <1 y – may be considered entirely normal
o Older children – may be a symptom of GOR or GORD
Explain the aetiology of GORD
• Results from transient lower oesophageal sphincter relaxation (due to immaturity in early age)
• Several anatomical + physiological features make infants <1 more prone to GOR than older children and adults
o Short, narrow oesophagus
o Delayed gastric emptying
o Short, lower oesophageal sphincter that is slightly above, rather than below the diaphragm
o Liquid diet + high caloric requirement – putting a strain on gastric capacity
o Larger ratio of gastric volume: oesophageal volume
GOR epidemiology
- Premature birth
- Parental hx of heartburn or acid regurgitation
- Obesity
- Hiatus hernia
- Hx of congenital diaphragmatic hernia (repaired)
- Hx of congenital oesophageal atresia (repaired)
- Neurodisability (e.g. cerebral palsy)
GORD complications
- Failure to thrive
- Chronic lung disease
- Reflux oesophagitis
- Oesophageal strictures
- Recurrent aspiration pneumonia
- Recurrent acute otitis media (>3 episodes in 6 months)
- Dental erosion in a child with neurodisabiliy (e.g. cerebral palsy)
- Rarely, apnoea or apparent life-threatening events (episodes of combinations of apnoea, colour change, change in muscle tone, choking, gagging – sometimes considered “missed” SIDS)
GOR + GORD prognosis
• Usually begin <8w + resolve <1y of age in 90% of infants
• Improvement in regurgitation + GORF thought to occur because of
o An increase in the length of the oesophagus
o An increase in tone of the lower oesophageal sphincter
o A more upright posture
o A more solid diet
Impetigo transmission + causative agents
• Transmission occurs directly though close contact with an infected person or indirectly via contaminated objects e.g. toys, clothing, towels
• Bacteria enter the skin through breaks caused by minor trauma (e.g. insect bites, scratches) or underlying skin conditions (e.g. eczema, scabies)
• Non bullous impetigo – Staph aureus, Strep pyogenes or a combination
• Bullous impetigo – Staph aureus
o Bullae form when exfoliative toxins produced by S. aureus cause loss of cell adhesion in the superficial epidermis
Impetigo complications
- Acute glomerulonephritis (following streptococcal impetigo)
- Cellulitis
- Staphylococcal scalded skin syndrome
- Lymphangitis
- Osteomyelitis + septic arthritis
- Septicaemia
- Scarlet fever, urticaria, erythema multiforme following strep. Infection)
Impetigo prognosis
- Self-limiting condition
- Takes 2-3 weeks to clear if left untreated – abx treatment leads to more rapid resolution of infection + reduces the infective period
- Relapse – more common in people with underlying skin conditions (e.g. eczema) + in staphylococcal carriers
- In some cases (e.g. neonates, people with severe immunosuppression) – impetigo can be a serious condition leasing to life-threatening complications
Impetigo ddx
• Skin infections + infestations
o Bacterial skin infections – cellulitis, ecthyma, erysipelas, staphylococcal scalded skin syndrome, necrotizing fasciitis
Erysipelas – sharply demarcated erythematous plaques, typically unilateral + o n the face, oedema, warmth
o Fungal skin infections – candidiasis, tinea corporis, tinea capitis
Dermatophytosis (tinea…) – peripheral scale, central clearing, crusting infrequent
o Parasitic infestations – scabies
Scabies – dermoscopy showing the classic burrow found particularly on the hands, elbows, genitals
o Viral infections – VZV, HSV
• Non infective skin conditions
o Dermatitis – atopic, contact, herpetiforms
Dermatitis herpetiforms – Associated with coeliac disease – multiple vesicles grouped on the lower back + around elbows + knees, intensely puritic
o Insect bites
o Bruns and scalds
o Drug reactions
o Other skin disorders – pemphigus vulgaris, bullous pemphigoid, lupus erythematosus, erythema multiforme, sweets syndrome, SJS
Pemphigus vulgaris – larger superficial blisters, prominent mucosal involvement, older patients
Erythema multiforme – target lesions with violaceous centre, pt uncomfortable
SJS – involvement of the conjunctiva, pt systemically ill
What is Kawasaki disease?
- Vasculitis affecting medium sized arteries
- Acute febrile illness lasting >5 d
- Assess children with fever lasting >5 d for Kawasaki disease
- Not contagious
Diagnosis of Kawasaki disease
For diagnosis
• Fever
Plus 4/5 of
• Polymorphous erythematous rash
• Non-purulent bilateral conjunctival injection
• Oropharyngeal changes (incl. diffuse hyperaemia, strawberry tongue, lip changes)
• Peripheral extremity changes (incl erythema, oedema, induration, desquamation)
• Non purulent cervical lymphadenopathy
Ask parents about the presence of these features since the onset of fever – may have resolved by the time of the assessment
Incomplete Kawasaki disease – fever but not enough other features to fit the dx criteria (15-20%) – increased risk of complications due to diagnostic delay
Kawasaki disease complications + prognosis
• Coronary aneurysm – in 20-25% of untreated patients o Regress after 1-2 years o 1% become giant aneurysms (>8mm diameter) • Sudden cardiac death • MI • Coronary artery aneurysms + rupture • Pericarditis • Myocarditis • Cardiac valvular disease • Cardiac dysrhythmia • Heart failure • Acute arthritis • Dehydration in the acute phase of the illness
- 0.08-3.7% mortality
- Leading cause of acquired heart disease in children <5
- Up to 50% show echo evidence of cardiac impairment + mild MR
- Recurrence in <1%
Kawasaki disease ddx
• Staph or strep infection
• Systemic JIA – syndrome of fever, rash, lymphadenopathy, arthritis
• Scarlet fever
o URTI, diffuse papular erythematous rash on trunk/extremities/face with circumoral pallor
o Resolution of rash associated with desquamation that starts in the face and progresses downward
o Unlike KD – in scarlet fever lops are spared + there is no conjunctivitis
• Acute rheumatic fever
• Toxic shock syndrome
• Staphylococcal scalded skin syndrome
• SJS – high fever, pronounced constitutional symptoms, skin rash manifested by diffuse bullae, involvement of mucosal membranes
• TEN
• Drug reaction
• Measles – exudative conjunctivitis, Koplik’s spots, rash that typically begins behind ears, patients appear more unwell
• Rubella
• EBV
• Parvovirus B19 infection
• Enteroviruses
• Meningitis or encephalitis
Normal puberty characteristics
o Acquisition of secondary sexual characteristics
o Accelerated linear growth
o Increase in the secretion of sex hormones
o Maturation of gonads (testes in boys, ovaries in girls)
o Potential for reproduction
o Typically complete within 2 to 5 years
o For boys begins when tetes size is at stage 4 on Prader orchidometer
Precocious puberty definition
• Precocious puberty = Appearance of secondary sexual characteristics
o <8 years in girls
o <9 years in boys
• 2 forms
o Gonadotrophin dependent precocious puberty (GDPP)/Central precocious puberty – due to premature activation of the hypothalamo-pituitary-gonadal axis
o Gonadotrophin independent precious puberty (GIPP)/precocious pseudopuberty or peripheral precocious puberty– autonomous secretion of sex steroids
Causes of gonadotrophin dependent precocious puberty GDPP
o Typically idiopathic
o Abnormalities of the CNS
Tumours – gliomas, astrocytomas, hamartomas, pineal tumours, hcg-secreting germ cell tumours can provoke premature activation of the hypothalamo-pituitary-gonadal axis
• Most common – optic + hypothalamic gliomas, astrocytomas
• Hamartomas of the tuber cinereum – congenital tumours composed of a heterotopic mass incl. GnRH neurosecretory neurones GDPP often occurring <3y, particularly in males
CNS trauma or injury – infection, radiation, surgery
Hamartomas of the hypothalamus
Congenital disorders – hydrocephalus, arachnoid cysts
o Sexual abuse – precipitating cause
Causes of gonadotrophin independent precocious puberty GIPP
o CAH
21-hydroxylase
Males – GIPP
Females – virilisation (pubic + axillary hair, clitoromegaly) due to excess androgen, no breast development
o Tumours – HCG-secreting tumours of the liver (hepatomas, hepatoblastomas), choriocarcinomas of gonads/pineal gland/mediastinum, adrenal tumours, ovarian tumours, testicular Leydig-cell tumours
High sex steroid concentrations
Leydig cell tumours – associated with virilisation + conversion of testosterone estradiol leads to gynaecomastia in males
Gonadoblastomas, adrenal virilising tumours
Granulosa cell + germ cell tumours – can secrete both androgens + estradiol
Hcg gonadotrophin-secreting germ cell tumours – may occur in the gonads, brain, liver, retroperitoneum, posterior mediastinum – are rare and cause precocious puberty in males only
o McCune-Albright syndrome
Risk of multiple endocrinopathies – thyrotoxicosis, Cushing’s, acromegaly, hyperparathyroidism
Café au lait spots, pathological fractures (fribrous dysplasia of the bones), recurrent ovarian cysts
More common in girls
o Testotoxicosis – in the first 2-3 years of life
o Severe hypothyroidism/ van Wyk-Grumbach syndrome – growth is arrested (unusual with precocious puberty) rather than accelerated
o Exogenous oestrogen or androgen exposure (therapeutic or accidental)
Precocious puberty complications
• Short stature as an adult
o Sex hormones directly stimulate the growth plate + GH secretion (from oestrogen from ovaries or aromatised form of testicular testosterone) growth spurt
o Paradox of tall stature in childhood due to accelerate rate of linear growth
o Normal/short stature as adult early fusion of the epiphyseal growth plates
• Psychological problems
Precocious puberty prognosis
• Reduced adult height due to accelerated skeletal development
o Treatment with GnRH improves adult height, particularly in <6
o If >7 – treatment may have little effect on adult height
o For GDPP without treatment, most girls aged 6-8 years at the onset of puberty will achieve adult height within the normal range
• In patients with long-standing GIPP, prolonged exposure to high levels of sex steroids can lead to GDPP (sex steroids may have a direct maturational effect on the hypothalamus + accelerate the onset of centrally mediated puberty)
• Gonadotrophin secretion recommences approx. 3-4 months after stopping treatment, with normal pubertal progress + fertility
Precocious puberty ddx
• When there is rapid progression of signs and accelerated growth – rule out CAH + virilising adrenal tumours
• Premature thelarche
• Premature adrenarche
• CAH
• Adrenal tumours
o Short hx of virilisation, accelerated growth rate, advanced bone age
o Ix – urine steroid profile analysis (abnormal pattern of adrenal hormone secretion), imaging of adrenal glands
• Cushing’s syndrome
o Virilisation without testicular enlargement/breast enlargement
o Cushingoid features – central obesity, thin extremities, nuchal fat pad, moon facies, purple striae, bruisability
o Ix - 24h urinary free cortisol, 8am serum cortisol with loss of circadian rhythm, undetectable ACTH, low + high dexamethasone suppression test (failure of cortisol production)
• PCOS
• Primary hypothyroidism
o high TSH can cause high FSH – isolated breast development/testicular enlargement without other secondary sexual characteristics
o no pubertal progression in the majority of cases
o delayed bone age + poor growth velocity
o TSH directly activates the FSH receptor (the 2 hormones have structural similarity)
CAH cause
• Family of inherited enzyme deficiencies that impair normal corticosteroid synthesis by the adrenal cortex
• Most common enzyme deficiency – 21-hydroxylase deficiency (90%)
o Classical CAH
- Production of cortisol occurs in the zona fasciculata
- Deficiency of 21-a hydroxylase insufficient cortisol production production of CRT + ACTH
- High ACTH adrenal hyperplasia + production of excess androgens which do not require 21-hydroxylase synthesis
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CAH types
o Classical CAH
Salt-wasting (75%)
• Most severe form of disease
• Symptoms of hyperandrogenism
o May present with atypical genitalia in females, hyperpigmentation in males
• Renal salt-wasting
o Characterised by GC + MC deficiency life-threatening adrenal crises + salt-wasting crises – vomiting and dehydration, occur early in infant life
Simple virilising (25%)
• Enzyme defect is moderate
• Females – Dx at birth – clitoral enlargement, partially fused labia majora, urogenital sinus
• Males – differentiation of external genitalia unaffected, may only present with hyperpigmentation (suggest ACTH)
• Sign of hyperandrogenism
• Retain ability to conserve salt
o Non-classical
Mild to moderate enzyme deficiency
Present in later childhood or early adulthood with signs of hyperandrogenism
Females do not have virilised genitalia at birth
Signs of hyperandrogenism
May present as precocious puberty (axillary hair or odour, pubic hair, acne, tall stature with advanced bone age that may eventually result in short stature)
Females – Oligomenorrhoea, amenorrhoea, delayed menarche, PCOS, acne, hirsutism, alopecia, impaired fertility, temporal baldness
Males – early beard growth, enlarged phallus, small testes, short stature, oligozoospermia
CAH complications + prognosis
• Iatrogenic Cushing’s syndrome
• Adrenal crisis
o Due to decreased cortisol levels
o Azotaemia, vascular collapse, shock, death
o can occur as early as 1-4 weeks of age
o mx – IM or IV hydrocortisone + IVF
• Short stature
• Osteopenia + fracture risk
o Due to long term GC use
• Testicular adrenal rests
o Benign tumours seen in male patients with classical salt-wasting CAH who are inadequately treated
o Deficient spermatogenesis
o Regular screening with testicular US, beginning in adolescence
o MRI or US + biopsies to confirm the benign nature of the tumour
o GC replacement will cause reduction in the masses
o Testis sparing surgery or orchidectomy may be required
• Precocious puberty
• PCOS
• Infertility
• Common – obesity, insulin resistance, hypertension - risk of CVD
Prognosis
• Healthy, normal lives if adherent to treatment
• Poor adherence - hyperandrogenaemia, Addisonian crisis
• Chronic glucocorticoid therapy – adrenal insufficiency
• Virilisation of female infants born to mothers with CAH has not been reported but is a possibility in uncontrolled cases.
CAH ddx
• Addison’s disease
o Muscle weakness, fatigue
o ACTH stimulation in Addison’s disease poor or absent cortisol response
• PCOS
• Gender dysphoria – lab tests normal
• Familial glucocorticoid syndrome
o Pallor, sweating, palpitations, hunger, visual changes, mental status changes
• Renal salt-wasting
o Hx of diuretic use and/or uncontrolled DM
o 17-hydroxyprogesterone is normal
Delayed puberty definiion
• Lack of any pubertal signs by the age of
o 13 years in girls
o 14 years in boys
• May be
o Functional = constitutional delay, underlying chronic disease, malnutrition, excessive exercise
o Organic
Hypogonadotrohic hypogonadism = Lack of serum gonadotrophin production or action
• Usually due to a hypothalamo-pituitary abnormality
• Hypothyroidism
Hypergonadotropic hypogonadism = gonadal insufficiency with elevated gonadotrophins
• E.g. Turner syndrome, Klinefelter’s syndrome
Delayed puberty RF
- FHx of delay
- Congenital pituitary abnormalities
- Gene mutations
- Chromosomal disorders
- Syndromic dx
- Anosmia
- Eating disorders
- Chronic systemic illness
- Malnutrition
- Intense exercise
- Congenital + acquired gonadal abnormalities
Delayed puberty complications
- Osteoporosis – absence of sex steroids inadequate bone mineralisation
- Psychological problems
- Skin irritation from gels and patches
- Polycythaemia – particularly with IM testosterone preparations
Delayed puberty prognosis
- Temporary delay e.g. constitutional delay – excellent prognosis, achieve normal gonadal function post-puberty without testosterone or oestrogen replacement therapy
- Chronic illness/malnutrition/intense exercise – recover after resolution of illness or exercise
- Permanent cause – organic gonadotrophin deficiency, Turner’s, Klinefelter’s, previous pituitary surgery for craniopharyngioma – lifelong hormone therapy
Delayed puberty ddx
• POI or Premature testicular failure
o Pt may have had normal pubertal development or present with pubertal arrest
• Hypothyroidism
o Causes hypogonadotropic hypogonadism leading to delayed puberty
• PCOS
• Outflow tract obstruction (incl. imperforate hymen, transverse vaginal septum) - normal pubertal development and lack of menarche – O/E either a perirectal mass or bulging hymen with haematocolpos
• Complete androgen insensitivity
o Phenotypically female with normal timing of breast development – 46XY male in a phnoypic female
o Minimal to no pubic hair growth, no menarche
o O/E – absent/blind vaginal pouch, palpable inguinal mass (testes)
o US – presence of testes with no ovaries or uterus
• 5-a reductase deficiency
o Boys – Poor virilisation at puberty
o Genital ambiguity
o Testosterone: dihydrotestosterone ratio markedly elevated
• Cushing’s syndrome
o May present with oligomenorrhoea
o Normal pubertal development
Define Juvenile idiopathic arthritis
• A collection of chronic paediatric inflammatory arthritides characterised by
o onset <16 years of age
o the presence of objective arthritis (in >1 joints)
o for at least 6w
o other known conditions are excluded
• There are several subtypes
o Oligoarticular
o Polyarticular
o Systemic onset
• A syndrome of fever, rash, lymphadenopathy, arthritis
• Fever of unknown origin, organomegaly, serositis (pericarditis, pleuritis)
• Anaemic patients, extremely high acute-phase markers
• Arthritis of joints = swelling or effusion, warmth and/or painful limited movement +/- tenderness
