paeds take 2 Flashcards
Is bronchiolitis an upper or lower RTI?
Lower
Most common viral cause of bronchiolitis
RSV
Age group affected by bronchiolitis
Age 1-9 months
RF for bronchiolitis
- Brest fed <2 months
- Smoke exposure
- Siblings
- Chronic lung disease due to prematurity
Bronchiolitis pathophysiology
- Proliferation of goblet cells –> excess mucus production
- Inflammation, bronchiolar constriction
- Lymphicytes –> submucosal oedema
- Mucus, oedema and increased cells in bronchioles –> hyperinflation, increased airway resistance, atelectasis and VP mismatch
bronchioles inaflame, produce mucus, oedema and necrosis cause obstruction
S+S of bronchiolitis
- Coryzal Sx precede cough and breathlessness
- Dry wheezy cough
- Cyanosis
- Tachypnoe and tachycardia
- Recession
- Hyperinflation
Ix bronchiolitis
- Swabs
- Urine and blood if pyrexic
- Examination
When to admit with bronchiolitis
- Apnoea
- <92%
- <70% normal drinking volume
- Severe resp distress
Bronchiolitis Mx
- Supportive
- Oxygen
- Fluids if dehydrated
- CPAP if struggling to breathe
Croup
Common viral childhood illness
Age of croup
6 months to 6 years
Most common croup organism
Parainfluenza virus
S+S croup
- Few days onset
- Coryza preceding
- Severe barking cough
- Harsh rasping stridor
- Worse at night
- Temperature
Ix croup
CLinical diagnosis
Mx croup
- All children with mild, moderate or severe - single dose or oral dexamethasome
- Can be managed at home if mild
- Severe upper airway obstruction = nebulised epinephrine with oxygen facemask
Neonates pneumonia organisms
GBS
E coli
Klebsiella
S aureus
Infants pneumonia organisms
S pneumoniae
Chlamydia
whooping cough mx
- exclude 48hrs from abx or 21d onset
- <1m old = clarythromycin 1st pr axith
- 1-12m = azith and clarith
- > 1y = azith and clarith
School age oneumonia causes
S pneumoniae
S aureus
GAS
M pneumoniae
Intussusception definition
Invagination of proximal bowel into distal segment
S+S intussusception
- Paroxysmal severe colicky pain with pallor
- Pale around mouth, draw up legs
- Recovery but increased lethargy between episodes
- Vomit may be bile stained
- Sausage shaped palpable mass
- Redcurrant jelly = blood stained mucus
Mx intussucpetion
Fluid resus
Air insufflation
Surgery
meckels diverticulum
- remnant of omphalomesenteric duct
- 2 feet from IC valve, 2 inches long
- rectal bleeding = painless
99 t scan
surgery
Pyloric stenosis S+S
Non bilious vomiting after feed, increasing in frequency and forcefullness until projectile
- Keep feeding
- Gastric peristalsis
- Pyloric mass = palpable during feed in RUQ
Pyloric stenosis Ix
- Test feed with NG tbe to aspirate stomach and exam
- USS
Mx pyloric stenosis
- Fix acid base electrolyte imbalances before surgery
- Pyloromyotomy
GORD definition
Involuntary passage of gastric contents into the oesophagus
Aetiology GORD
functional immaturity of lower oesophageal sphincter = inappropriately relaxed
RF for GORD
Prematurity
CP
Obesity
HH
S+S GORD
- Faltering growth from severe vomiting
- Oesophagitis
- Aspiration, wheezing, hoarseness
- Dystonic neck posturing
- Apnoea
Ix GORD
- Ph monitoring
- Endoscopy
- Barium swallow
Mx GORD
- Feed thickening
- PPI
- Antacid, H2 blocker
- Fundoplication
Kawasaki definition
Systemic vasculitis with a predisposition to involving coronary arteries
Diagnostic criteria Kawasaki
Fever >5 days and 4/5 of
- Bilateral, bulbar, non purulent conjunctivitis
- Changes in lips/oral mucosa = cracked, strawberry tongue, erythema
- Changes in extremities = Oedema, erythema, desquamination
- Polymorphous rash
- Cervical lymphadenopathy
Ix Kawasaki
- CLinical findings
- High inflam maekers
- Platelet count rises in 2nd week
Mx kawasaki
- IVIG 2g/kg 12hr apart
- IV methylprednisolone and infliximab
- Aspirin
- Antiplatelet agents of risk of thrombus
- Cardiology follow up
Measles features
- Temperature
- Rash = behind ears to body
- Kolpik spots
- Conjunctivitis and coryza
- Cough and malaise
Measles treatment
- Supportive
- Immunocompromised = ribavirin
Nephrotic syndrome definition
- Proteinuria, hypoalbuminaemia, oedema
Nephrotic pathophysiology
- Podocytes flattened so allow leaking
S+S nephrotic syndrome
Oedema = initially on waking, periorbital
- Ascites, labial and scrotal swelling
- Pleural effusions and breathlessness
Nephrotic Ix
- Urinalysis
- Microscopy
- Serum albumin
- U+E/creatinine
Nephrotic Mx
- Oral corticosteroids (60mg/m2 per day pred) –> 4 weeks change to 40 on alternate days
- Resistant = diuretics, salt restriction, NSAIDs
Nephrotic indications for biopsy
- <12 months or >12 years
- Increased BP
- Macroscopic haematuria
- Impaired renal function
- Decreased C3/C4
- Failure to respond after 1 month daily steroid therapy
S+S VSD
- Small = asymptomatic
- Pan systolic murmur lower left sternal edge
- HF after 1 week if large (diuretics and captopril)
- Tachycardia, pnoea and hepatomegaly
4 cardinal features of TOF
- Large VSD
- Overriding aorta
- Right ventricular hypertrophy
- Sub pulmonary stenosis causing RV outflow tract obstruction
pulmonary stenosis finding
ESM upper left sternal border
S+S TOF
- Cyanosis
- Paroxysmal hyper cyanotic spells
- Ejection systolic murmur
Definition of rheumatic fever
Multisystem autoimmune response to a group A strep infection
Major criteria for RF
- Subcutaneous nodules
- Erythema marginatum
- Chorea (sydenham)
- Migratory arthrotos = ankles, knees, wrists
- Carditis
Minor criteria for RF
- Fever
- Polyarthralgia
- Raised CRP/ESR
- Prolonged PR
need 2 makor or 1 major and 2 minor
Rheumatic Fever Mx
- NSAIDs
- Glucocorticoids if severe carditis
- Penixillin
- Assess for emergency valve replacement
Direction of shunt in PDA
- Left to right
- Flow of blood from aorta to PA following fall in PV resistance after birth
S+S PDA
- Continuous murmur below left clavicle = machinery
- Collapsing/bounding pulse
- If large = poor growth, feeding difficulty, resp difficulty, tachypnoea
- Thrill/gallop
S+S IE
- Sustained fever, malaise, raised ESR, unexplained anaemia or haematuria = suspect
- Murmur/changing cardiac signs
- Anaemia and pallor
- Nail signs
- Necrotic skin lesions
- Splenomegaly
- Neuro signs from cerebral infarction
Major Dukes criteria
- +ve blood culture = 2 seperate
- Evidence of endocardial involvement = +ve echo fidnings
Minor dukes criteria
- Predisposition
- FEver
- Vascular phenomena
- Immunologic phenomena
- Microbiological evidence
- ECHO
IE treatment
- High dose enicillin and aminoglycoside
- 6 weeks OV
HSP definition
Small vessel non-granulomatous IgA leukocytoclastic vasculitis
RF HSP
- URTI
- Winter
- 2-11
HSP patho
Circulating IgA levels increase and IgG synthesis disrupted. IgG and A produce complexes that activate complement and are deposited in affected organs
HSP S+S
- Maculopapular purpuric palpable rash = legs, buttock, arms- Trunk spared
- Oedema
- Abdo colic pain (haem and melaena)
- Arthralgia
- Macrscopic haematuria
- Severe = intussuception
HSP Tx
- Steroids
- Benign and self limiting = most recover in 8 weeks
Glomerulonephritis definition
Acute renal failure due to damage/injury of glomeruli
Causes GN
- Postinfectious
- Vasculitis
- IgA nephropathy
- Goodpastures
S+S GN
- HTN –> seizures
- Oedema
- Reduced UO and volume overload
- Haematuria and proteinuria
Ix GN
- Urine dip
- Microscopy = RBC and protein casts
- USS
Mx Gn
- Treat hyperK, HTN, acidosis, an hypoca
Poststrep GN
- Throat or skin infection
- Low complement C3 levels return normal after 4w
- Culture to prove
IgA GN
- Macroscopic haematuria
- URTI
Alport syndrome
- Abnormalities of collagen in BM
- X linked recessive
- Nerve deafness and ocular defects
Triad for nephrotic
- Heavy proteinuria
- Low plasma albumin
- Oedema
Vesicoureteric reflux
Ureters displaced laterally and enter directly into bladder rather than at an angle
Long term impact vesicoureteric reflux
- Urine returning to bladder from ureters after voiding = incomplete empyting = infection
- Pyelonephritis
- Bladder voiding rpessure = transmitted to renal papillae = renal damage
Triad of HUS
- Microangiopathic haemolytic anaemia
- Thrombocytopenia
- AKI
HUS S+S
- fever
- abdo pain
- lethargy
- oliguria
- haematuria
- bruising
- jauncide
- confusion
Mx HUS
- IV fluids
- Transfusions
- DIalysis
self limiting = most fully recover
- Early supportive therapy and dialysis
Colic
- Inconsolable crying
- Drawing up knees
- Excessive wind
- Evening
- Benign
- > 2 weeks = cows milk allergy
Toddlers diarrhoea
- ‘Peas and carrots’
- Well and thriving children
- Adequate fat and fibre
Whooping cough
- Bordetella pertussis
- 1 week of coryza develop paroxysmal or sporadic cough
- Inspiratory whoop
- Night worse
- Vomiting
- Red or blue in face and mucus
- 3-6 weeks
- PCR = marked lymphocytosis on blood film
- Immunisation
Slapped cheek
- Erythema infectiosum
- viraemic phase of fever, malaise, headache and myalgia
- rash on face turns to lace like maculopapular rash on trunk and limbs
Candida
- May cause and often complicates napkin rashes
- Erythematous
- Skin flexures and may be satellite lesions
- Topical antifungal
Breath holding episodes
- Toddler
- Precipitated by anger
- Goes blue then limp
- Rapid recovery
Reflex anoxic seizures
- Toddler
- Precipitated by pain
- Stops breathing
- Goes pale
- Brief seizure
- Rapid recovery
Epilepsy
- Same as adults so look at treatment
Juvenile myoclonic epilepsy
- Myoclonic seizures shortly after waking
- Throwing drinks or cornflakes
West syndrome
- Infantile spasms
- 6 months age
- Clusters of full body spasms
- Prednisolone and vigabatrin
undescended testes
- 4% at birth
- Preterm more common
- Retractacile = can be manpulated but then retracts
- Palpable = can feel but not manipulate
- Impalpable = no testis felt
Ix = USS, measure testosterone after HCG injections, laparosciopy
Mx = surgery
testicular torsion
- Atypical presentation if young with lower or inguinal abdo pain of sudden onset
- Surgical exploration mandatory
Definition haemolytic disease of new-born
- Caused by incompatibility between rhesus antigens on surface of RBCs of mum and foetus
- Mum Rh- and baby Rh+. 1st pregnancy mum will become sensitised to RhD antigens so subsequent pregnancies = anti D abs cross placenta = haemolysis = anaemia and high bilirubin levels
- DCT
Microcytic anaemia causes
Thalassaemia
Anaemia chronic disease
Iron def
Lead poisoning
Sideroblastic
TAILS
~~~
Thalassaemia definition
- A thal = defect in A globin chains
B thal = defect in B globin chains - Autosomal recessive
S+S thalassaemia
- Microcytic anaemia
- Fatigue and pallor
- Jaundice
- Gallstones
- Splenomegaly
- Pronounced forehead
- Poor growth
Thalassaemia diagnosis
- FBC = microcytic anaemia
- Hb electrophoresis
- DNA test
G6PD deficiency
- X linked
- Neonatal jaundice
- Acute haemolysis = fever, malaise, dark urine
diagnosis and Mx G6PD
- Measuring G6PD activity in RBC
- Avoid precipitants
Sickle cell inheritance
- Autosomal recessive
- 1 abnormal = trait
- 2 abnormal = disease
- Sickle cell anaemia = homozygous
manifestations of SCD
- Anaemia and jaundice
- Increased susceptibility to infection
- Vaso-occlusive crises
- Acute anaemia
- Splenomegaly
SCD Mx
- Antibiotic prophylaxis
- Folic acid
- Hydration
- Crises = analgesia, hydration, abx, exchange of transfusion
Haemophilia definition
- X linked recessive
- A = FVIII deficiency
- B = FIX
S+S haemophilia
- Recurrent spontaneous bleeding into joints and muscles
- Neonates = IC haemorrhage, prolonged bleeding from heel stick
Haemophilia management
- Recombinant FVIII or FVIX concentrate IV infusion if bleeding
VWD
- deficiency of VW factor = defective platelet plug formation
- Autosomal dominant
S+S VWD
- bruising
- prolonged bleeding
- Mucosal bleeding
VWD Mx
- Desmopressin
- VWF
- factor VIII
ITP
- Caused by type 2 sensitivity reaction
- Antibodies target and destroy platelets
ITP presentation
- Under 10
- Recent viral illness
- Bleeding and bruising
- purpuric non blanching rash
- DO an urgent FBC for platelet count
ITP Mx
- Prednisolone
- IVIG
- Transfusions
- Platelets
Avoid in ITP
- Contact sports
- Avoid IM injections
- Avoid NSAIDs, aspirin and blood thinners
What leukaemia is most common in children
ALL
Presentation ALL
- 2-5 years
- Malaise
- Anorexia
- Anaemia
- Neutropenia
- Pallor
- Bruising, nosebleeds
- Headaches, vomting, palsies
Ix ALL
- FBC (low Hb, TP)
- Blood film
- BM biopsy
- CXR
Mx ALL
- Chemotherapy
Neuroblastoma Ix
- Raised urinary catecholamine levels
- USS and MRI = abdo mass
Neuroblastoma Mx
- Surgery if localised
- Metastatic = chemo
Wilms tumour
- Neuroblastoma
- From embryonal renal tissue
S+S Wilms
- Large abdo mass
- Anaemia, haematuria, HTN
Wilms Ix and Mx
- USS or CT/MRI = intrinsic renal mass
- Initial chemo followed by delayed nephrectomy
Bone tumour
- Osteogenic sarcoma more common than Ewing
- Ewing in younger children
- Male predominance
- Bone pain
- XR
- Chemo and surgery
Retinoblastoma
- Malignant tumour retinal cells
- Csome 13 = susceptibility gene = dominant
S+S retinoblastoma
- white pupillary reflex to replace red reflex
- Squint
Ix and Mx retinoblastoma
- Enucleation if advanced
- Chemo t shrink
- Local laser treatment
Downs syndrome features
trisomy 21
- Hypotonia
- Brachycephaly
- Short neck
- Short stature
- Flattened face and nose
- Prominent epicanthic folds
- Upward sloping palpebral fissures
- Single palmar crease
Downs syndrome complications
- Learning disability
- Recurrent otitis media
- Deafness
- Visual problems
- Hypothyroid
- Cardiac defects
- Leukaemia
Klinefelter syndrome
- Male has additional X csome = 47XXY
Mx klinefelter
- Testosterone injecitions
- MDT input
Turner syndrome
- Female has single X csome = 45X0
Turner features
- short stature
- Webbed neck
- High arching palate
- Ptosis
- Broad chest
- Cubitus valgus
- Underdeveloped ovaries
- Late or incomplete puberty
Turners Mx
- GH therapy
- Oestrogen and progesterone
Features fragile X
- Intellectual disability
- Long narrow face
- Large ears
- Large testiles
- Hypermobile joints
- ADHD
- Autism
- Seizures
- Mitral valve prolapse
Prader Willi features
- Csome 15 = deletion
- Insatiable hunger
- Hypotonia
- Hypogonadism
- Soft fair skin
- MH problems
- Narrow forehead
- Strabismus
- Thin upper lip
Patau syndrome
- Trisomy 13
- Rocker bottom feet
- polydactyly
- cleft lip and palate
- microcephaly
RDS definition
- Surfactant deficiency = lowers surface tension = alveolar collapse and inadequate GE
How to reduce risk of RDS
- Glucocorticoids gien antenatally to mother to stimulate foetal surfactant production
S+S RDS
- Within 4hrs of birth will get
- Tachypnoea >60
- Laboured breathing with chest wall recession and flaring
- Expiratory grunting
- Cyanosis
RDS CXR
- Ground glass appearance and air bronchogram
RDS Mx
- Raised ambient oxygen
- May need continuous positive airway pressure or artificial ventilation
Necrotising enterocolitis
- Bacterial invasion of ischaemic bowel wall
S+S NE
- Stop tolerating feeds
- Milk aspirated from stomach
- Vomiting (bile)
- Abdo distension
- Blood in stool
- Shock
NE XR
- distended bowel loops and thickening of bowel wall with intramural gas
Mx NE
- Artificial ventilation
- Stop oral feeding
- Broad spectrum abx
- Parenteral nutrition
- Surgery if perforation
Kernicterus
- Encephalopathy resulting from deposition of unconjucated bilirubin in basal ganglia and brainstem nuclei
What level to babies become clinically jaundiced
- 80 umol/l
Jaundice <24 hrs age
- Usually results from haemolysis
- Bili = unconjugated and can rise rapidly
Haemolytic disorders
- Rhesus haemolytic disease
- ABO incompatibility
- G6PD
- Spherocytosis
Causes of jaundice 2 days - 2 weeks
- Physiological
- Breast milk jaundice
- Dehydration
- Infection
Management of jaundice
- Phototherapy
- Exchange transfusion
Jaundice >2 weeks old
- Persistent neoneatal jaundice
- May be caused by biliary atresia
Meconium aspiration
- Asphyxiated infants may start gasping and aspirate meconium before delivery
- Meconium = lung irritant = mechanical obstruction and chemical pneumonitis
- Overinflated lungs with patches of collapse and consolidation
- Pneumothorax
- May develop persistant pulmonary HTN
S+S neonatal sepsis
- Fever
- Poor feeding
- Vomiting
- APnoea and bradycardia
- RDS
- Abdo distension
- Jaundice
- Neutropenia
- Glycaemia
- Shock
- Seizures
Group B strep infection RF
- PPRM
- Maternal fever in labour
- Maternal chorioamnionitis
- Previous infected infant
- Prophylactic abx given
Hypoglycaemia symptoms
- Jitteriness
- Irritability
- Apnoea
- Lethargy
- Drowsiness
- Seizures
Ix neonatal seizre
- Cerebral USS
- Hypoglycamia and meningitis need to be ruled out
Gastroschisis
- Bowel protrudes through defect in anterior abdominal wall adjacent to umbilicus
- Risk of dehydration and protein loss
- Clingfilm to minimise fluid and heat loss
- NG tube
- IV dextrose
- Colloid support
- Surgerhy
Foetal problems associated with maternal diabetes
- Congenital malformations
- IUGR
- Macrosomia
Pathophysiology T1DM
- Destruction of pancreatic B cells by autoimmune process
S+S DM1
- Polydipsia
- Polyura
- Weight loss
- Candida
- Sking infection
- DKA
- 2 peak ages = age 5-7 and before puberty
Ix DM1
- Random glucose >11.1
- U+E, ketones, urine, blood pH, autoantibodies
- Fasting blood glucose >7
Mx DM1 insulin changes on age
Requirement for insulin changes on age
- Childhood = 0.5-1 u/kg/day
- Puberty = 1.2 - 2 kg/day
- post puberty = 0.7-1.2 u/kg/day
Target = 4-10mmol/l
S+S DKA
- Acetone breath
- Vomiting
- Dehydration
- Abdo pain
- Kussmaul
- Hypo shock
- Drowsy
Definition DKA
- Hyperglycaemia >11mmol/l
- pH <7.3
- Bicarb <15
- Urinary ketones
DKA Ix
- Glucose
- Ketones >3
- ABG
- U+E, creatinine
- Cardiac monitor
- Weight
- Lactate
ABG results for mild, moderate and sever DKA
- Mild = pH >7.2 or HCO3 10-15mmol/l
- Moderate DKA = pH 7.1-7.2 or HCO3 5-10
- Severe DKA = pH<7.1 or HCO3 <5
Mx DKA
- Emergency measures = if shocked give bolus 10ml/kg 0.9% NaCl and cosider abx
- Fluids = no shock give initial 10ml/kg bolus +rehydration fluids + maintenance fluids
- Insulin 0.05 units/kg/hr
Complications DKA
- Cerebral oedema
- Rapid correction can cause rapid water shift from extra to intra = oedematous brain
Mx hypoglycaemia
- IV glucose 2ml/kg of 10% dextrose
- Sweating, pallor, CNS
Congenital hypothyroidism S+S
- Guthrie positive
- Prolonged neonatal jaundice
- Poor feeding
- Constiptation
- Increased sleeping
- Reduced activity
- Slow growth and development
Acquired hypothyroidism S+S
- Fatigue and low energy
- Poor growth
- Weight gain
- Poor school performance
- Constipation
- Dry skin and hair loss
CAH definition
- Underproduction of cortisol and aldosterone and overproduction of androgens from birth
- AR = congenital deficiency of 21 hydroxylase
CAH pathophysiology
- 21H converts progesterone to aldosterone and cortisol. lack therefore means extra progesterone converted to testosterone = low ald and high test
S+S CAH
- Ambiguous genitalia, enlarged clitoris
- Severe = after birth hypona, hyperka, hypogly
- Poor feeding
- Vomiting and dehydration
- Arrhythmia
older - Tall
- Facial hair
- Absent periods
- Small testicles, large penis
- Hyperpigmentation
Adrenal crisis S+S
- 1-3 weeks old
- Vomiting
- WL
- Floppy
- Circulatory collpase
Mx - Hydrocortisone
- Saline
- Glucose
CAH Ix
- Low plasma Na
- High plasma K
- Met acodosis
- hypo
Mx adrenal
- Hydrocortisone to replace cortisol
- Fludrocortisone to replace aldosterone
- Surgery
RF for OM
- 6-16 months
- Male
- Passive smoking
- Bottle feed
- Craniofacial abnormalities
suppurative = mucopurulent discharge
Effusion = grey TM, loss of light and fluid behind TM
S+S OM
- Severe pain
- Systemic features
- Coryzal
- Bright red and bulging tympanic membrane, loss of reflection
- Test facial nerve function
OM causative
- S pneumonia
- RSV, rhinovirus
Mx OM
- Usually spontaneous recovery
- ABX shorten pain but dont reduce hearing loss
- <2yo = antibiotics (amox, erythro)
OM complication
Mastoiditis
- Necrosis, subperiosteal abscess
- Boggy, erythematous swelling behind ear
- IV Abx
OM with effusion
- Glue ear = result of recurrent ear infection
- Viscous inflammatory fluid build up
- Eardrum dull, retraces, visible fluid level
- Asymtpomatic apart from hearing loss
- Spontaneous recovery
- Grommets if not recovering
S+S asthma
- Suspect in any child with wheeze
- Wheeze, cough and breathlessness worse at night and morning
- Interval symptoms
Mx asthma
- SABA for all
>5 - ICS
- LRTA
- LABA
- MART
<5 - ICS 8 week trial
- LRTA
Moderate asthma attack
- Talk
- Sats >92
- PF >50%
• RR - <40 for 1-5
- <30 for 5-12
- <25 for 12-18
• HR - <140 for 1-5
- <125 for 5-12
- <110 for 12-18
• SABA, prednisolone, monitor
Severe asthma attack
Too breathless to talk
•O2 sats <92 for <12
•Peak flow 33 – 50%
•RR
- >40 1-5
- >30 5-12
- >25 12-18
• HR
- >140 1-5
- >125 5-12
- >110 12-18
• Oxygen, SABA, prednisolone
• Consider ipratropium
Life threatening asthma attack
- Silent chest, cyanosis
- Poor resp effort
- Exhaustion
- Arrhhythmia, hypotension
- PF <33
- )2 <92
- Oxygen, SABA, Pred, neb ipratropium
Epiglottitis
- Similar clinical to croup
- H influenza
- Ill child, high fever
- Drooling unable to swallow
- Soft stridor
EMERGENCY - O2 and neb adrenaline
- Anaesthetist, paeds and ENT
- Keep calm as crying bad
CF definition
- AR = mutation in CF transmembrane condictance regulator fene
CF Pathophysiology
- Multisystem disorder = results from abnormal ion transport across epithelial cells
- CFTR gene encoded CFTR protein = Cl channel. Cl is driven against its concentration gradient using ATP
- Airways = reduction in airway surface liquid layer impaired ciliary function and retention of mucopurulent secretions which are prone to infection
- Defective CFTR = dysregulation of inflammation an defence against infection
= Intestine = thick viscid meconium meconium ileus in 10-20% - Pancreatic ducts = blocked with thick secretions pancreatic enzyme deficiency and malabsorption
- Sweat gland abnormal function excessive concentration of Na and Cl in sweat
CF S+S
- Meconium ileus
- Prolonged neonatal jaundice
- Recurrent infections
- Malabsorption, steatorrhea
- Bronchiectasis
- Persistent cough
- DM
- Cirrhosis and portal HTN
- hyperinflation, coarse creps inspiration and expiratory wheeze , clubbing
CF Ix
- Sweat test
- Genetic testing
- CXR = hyperinflation, bronchial dilation, cysts, linear shadows
CF Mx
- Nebulized mucolytics = DNase and hypertonic saline
- ABx
- Lactulose
- Pancreatic enzyme replacement
- CFTR modulators
- Physio
- high calorie high fat diet
JIA definition
- Persistent joint swelling of >6 weeks presenting before 16 YO in absence of infection or other cause
- Oligo = up to and including 4
- Poly = more than 4
S+S JIA
- Morning joint stiff
- Pain
- Young = intermittent limp or mood deterioration
- Swelling
- Gradual onset
- Non use
- Rash, fever, WL
Ix JIA
- Exclusion in children <16
- FBC, CRP/ESR, infection screen, Rh, ANA
- Imaging
- Early XR = soft tissue swelling, juxta-articular osteopenia
- Late XR = joint space narrowing and erosions
- Gadolinium enhanced MRO GS for synovitis
Mx JIA
- NSAIDs
- Joint injections = 1st line in oligo
- Methotrexate
- Corticosteroids if severe
- Biologics = cytokine modulators
JIA complications
- Chronic anterior uveitis = refer to opthalmology
- Joint contractures and erosions
DDH RF
- FHx
- Female
- Breech
DDH S+S
Infant
- Asymmetrical gluteal folds
- Limited abduction
- LL discrepancy
- Galeazzi sign
Older
- Limp
- +ve trendelenburg
- Bilateral dislocations = exaggerated lumbar lordosis and limited hip abduction
Ix DDH
- Ortolani and Barlow = NIPE
- <6 months USS
>6 months AP pelvis radiograph = shallow acetabulum, increased ondex, hypoplastic femoral head
DDH Mx
<6 months
- Pavlik harness
6-18 months
- Manipulation and closed reduction and plaster cast
18-24 months
- Trial of closed reduction +/- pelvic osteotomy and cast
2-6 years
- Open reduction +/- femoral shortening +/- pelvic osteotomy and cast
Perthes definition
- Idiopathic avascular necrosis of capital femoral head due to interruption of blood supply
RF Perthes
- Boys
- 5-10
- Obesity
- Trauma
- Endocrine/metabolic
S+S Perthes
- Insidious presentation
- Limp, hip or knee pain
- Mild intermittent anterior thigh/groin/knee pain
- Bilateral
- Painless limp
Ix perthes
- Early XR = normal
- Later XR = increased density in femoral head = fragmented and residual deformity
A = no loss of height of lateral 1/3
B = up to 50% loss of height
C = >50% loss of height - Technetium 99 scan
Perthes on examination
Look
- Proximal thigh atrophy
- Mild short stature
- Limp/Trendelenberg/antalgic gait
Feel
- Effusion
- Groin/thigh tenderness
Move
- Decreased hip ROM with spasm
Perhtes Mx
- Physio
- Rest and walking aids
- NSAIDs
- Surgery
- Local self healing disorder
Osteomyelitis definition
- Infection of metaphysis of long bone
Causative organisms Osteomyelitis
- Staph aureus
- Neonates = GBS
- <2 HI
- > 2 gram +ve
S+S osteomyelitis
- Low grade pyrexia and malaise
- Markedly painful immobile limb
- Tender, warm swelling
- POM
- Infants = swollen and ROM
Ix osteomyelitis
- Cultures
- WCC, CRP and ESR raised
_ XR = early normal, late = metaphyseal rarefaction, destructive changes after 10 days
Mx osteomyelitis
- IV Abx 2 weeks then oral for 4
- Drainage and debridement
Septic arthritis S+S
Knee>hip>ankle
- Erythematous warn tender joint
- Reduced ROM
- Hold limb still
- Joint effusion in peripheral joints
- 50% dont have fever
SA Ix
- Bloods = increased WCC
- Cultures
- XR = joint space narrowing and erosive changes
- Aspiration
- USS
- MRI
SA Mx
- IV Abx after aspirate taken for up to 3 weeks then oral 4-6 weeks
- Surgery = irrigation and debridement
- Splnitage
- Physio
Type 1 osteogensis imperfecta
- AD
- Fractures in childhood
- Blue sclera
Type 2 OI
- Lethal
- Multiple fractures before birth
S+S rickets
- Rachitis rosary
- Horizontal depression lower chest
- Bowed legs
- Softening of skull vault
- Delayed closure fontanelle
- Hypotonia
XR rickets
- Cupping and fraying of metaphyses
- Widened growth plate
- Joint widening
- Bowing of diaphysis
- Thickening and widening of epiphysis
Transient synovitis
- Follows or accompanied by viral infection
- Sudden onset in pain or a limp
- No pain at rest
- Decreased ROM
- May refer to knee
- Afebrile
Slipped upper femoral epiphysis
- RF = AA, endo, obese
- Groin thigh or knee pain
- Antalgic gait
- Limited hip flexion and abduction
- Thigh atrophy
Malrotation
- 1-3 days of life present with obstruction from Ladd bands obstructing duodenum or with volvulus
- Bilious vomiting, abdo pain, tender from peritonitis
- GI contrast study needed if bilious vomiting
- Surgical correction
Biliary atresia
- Section of bile duct narrowed or absent
- Cholestasis and prevented excretion conjugated bili
- Present after birth = jaundice (persistent) and high conj bili
- Surgery = kasai portoenterostomy
- Transplant liver
Pathophysiology cows milk allergy
IgE mediated
- Type 1 hypersensitivity
- CD4 + TH2 cells stimulate B to produce IgE abs = histomine and cytokines
Non IgE
- T cell activation againt CMP
IgE CMP S+S
- Acute onset
- Pruritus, erythema, urticaria, angio-oedema
- N+V
- Colick, diarrhoea
Non IgE CMP S+S
- Delayed
- Pruritus, erythema, atopic eczema
- GORD
- Loose stools
- Blood/mucus
- Abdo pain, food refusal, pallor, tiredness
Mx CMP allergy
- Avoidance
- MAP guideline
- Hydrolysed formula or AA formula
UC histology and radiology
- Crypt abscesses, mucosal inflammation, architectural distortion, crypt loss
- Mucosal ulceration, haustration loss, colonic narrowing
S+S UC
- Rectal bleed
- Diarrhoea
- Colicky
- Weight loss and grwoth failure
MX UC
- Amino salicylates for induction and maintenance
- Aggressive = steroids
- Surgery
Crohns patho
- Mouth to anus
- Skip lesions
- Transmural infalmmation = deep ulcers and fissures
- Non caseating granulomatous inflamation
Crohns S+S
- Growth failure, puberty delayed
- General ill health
- Abdo pain, diarrhoea, blood, weight loss
- Oral lesions
- Uveitis
Crohn’s Ix
- Bloods
PANCA - MC+S
- Calcoprotein
- Endoscopy and radiology (crypt abscesses, transmural inflammation, cobblestones)
Crohns Mx
- Mild = oral 5 ASA dimers
- Moderate = pred to induce remission
- Maintenance = Immunomodifiers
Crohn’s vs UC
Crohn’s
- Mouth to anus
- Transmural
- Discontinuous
- Granuloma and recr=tal sparing
- Fissures, fistula, strictures
- perianal disease
UC
- Colon only
- Mucosal
- Continuous
- no granuloma and no rectal sparing
- Abscesses and strictures rare
- primary sclerosing cholangitis
Gastroenteritis - general Ix
- Stool sample if blood, septicaemia, immunocompromised
U+E = IVF if hyperna
When to consider DD for gastroenterieis
- Temp >38 if <3m
- Tachy
- GCS altered
- Meningism
- Blood/mucus
- Green vomit
- Distension or guarding
Mx gastroenteritis general
- no dehydrated = feeds, fluids
dehydrated - fluid deficit replacement and maintenance
- IV if shock
- ORS 50ml/kg over 4 hrs
Classical form coeliac
- 9-24 months
- Malabsorption
- FTT and weight loss
- Loose stools and steat
- Anorexia
- Abdo pain and distension
- Behaviour change
- Crypt hyperplasia and villous atrophy
Atypical form coeliac
- No intestinal sx
- Extra sx = OP, neuropathy, anaemia, infertility
- +ve serology
- limited abnormalities
Hirschprung’s disease
- Absence ganglion cells in hindgut = ansence of coordinated peristalsis
- Neonates, fail mec in 24hrs, abdo distension and bile vomit
- older = chronic constipation, abdo distension, growth faltering
Hirschprung Ix and Mx
- DRE = gush stoll and flatus
- AXR = distal intestinal obstruction
- Biopsy
- Colostomy and anastomosis = Swenson procedure
When to biopsy for hirschprung
- delayed mecomium 48hrs
- constipation since first few weeks of life
- chronic distension and vomiting
- FHx
- Faltering growth
Diagnostic criteria for constipation
- <3 complete stolls a week
- hard large stool
- rabbit droppings
- overflow soiling
Constipation management
- laxative (macrogol) and diet
- stimulant laxative (senna)
- lactulose/softener
Measles
- Temperature
- rash = behind ears, whole body
- Koplik spots = white on inside cheeks
- Conjunctivitis, cough, malaise
- Supportive treatment
- immunocompromised = ribavarin
Mumps
- Fever, malaise,parotid gland swelling
- 1 side face swollen
- earache
- plasma amylase elevated
Rubella
- Maculopapular rash, lymphadenopathy
Complications - arthritis
- encephalitis
- thrombocytopenia
- myocarditis
Chicken pox
- VZV
- vessicles on trunk/face and spread outwards
- lesions scab over = no longer infectious
- fever, itch, malaise
- 5 day exclusion
- aziclovir if serious
- VZIG prophylaxis
Impetigo
- staph or strep skin infection
- lesions on facem neck and hands begin as erythematous papules
- rupture of vesicles = honey coloured crusted lesions
- topical hydroperoxidde abx
- fluclox if severe
toxic shock
- s aureus or group a strep
- fever over 39
- hypotension
- diffuse erythematou macular rash
- organ dysfunction
- thrombocytopenia, coagulopathy, abnomral LFTs
- manage shock, debridement
- cef with clindamycin
- IVIG to neutralise circulating toxin
Scalded skin syndrome
- Exfolaitive staph toxin
- fever, malaise
- Purulent crusting around eyes,nose, mouth
- widesread erythema and tenderness
- areas seperate ong ente oressure
- Fluclox, analgesia, hydration
HIV S+S
- lymphadenopathy
- parotid enlargement
- recurrent bacterial
- candidiasis
- chronic diarrhoea
- pneumonitis
HIV Ix
- > 18m = detect antibodies
- <18m = transplacental maternal IgG HIV antibodies so positive test shows exposure not infection
HIV Mx
- prophylactic co-trimoxazole if low CD4
- Immunisations
TB S+S
Asymptomatic
- Minimal or no S+S
- Positive Mantoux or interferongamma release assay = give chemoprophylaxis
Symptomatic
- Lung lesion plus LN = Ghon/primary complex
- Fever
- Weight loss
- Cough
- CXR changes
4-8 weeks
- Febrile illness
- Erythema nodosum
- Phlyctenular conjunctivitis
6-9 months
- Progressive healing of primary complex
- Effusion
- Cavitation
- Coin lesion on CXR
- Regional LN may obstruct bronchi
- Regional LN may erode into bronchus or pericardial sac
- Miliary spread
REACTIVATION = tuberculous meningitis
TB Ix
- Sputum on 3 consecutive = ZN stain for AFB
- Urine, LN, CSF
- CXR
- Mantoux skin test
Mx TB
pulmonary
- 2 months RIPE
- 4 months RI
miliary
- 3 months RIPE
- 12-18 months RI
EBV
- Causes mono
- fever, malaise, tonsilitis, petechia palate, maculopapuar rash
- +ve monospot test
- Symptomatic treatment
Febrile seizures
- 2-5minutes, high fever, underlying illness
- manage underlying source of infection
Hypospadias
- Urethral opening proximal to normal meatus
- Hooded dorsal foreskin
- Chordee
- No circumsision
- surgery before 2yo
HSP
- Non granulomatous IgA vasculitis
- URTI
- Purpura legs, bum, arms, symmetrical and trunk spared
- macroscopic haem or mild protien
- angioeoedema
- Mx = rest, analgesia, hydrate, steroids
Spina bifida occulta
- No herniation neural tissue
- dermal sinus, dimple, lipoma, hairy naevus
Spina bifida meningocele
- Herniation fluid and meninges only
skin covering - surgical closure
Spina bifida myelomeningocele
- Herniation spinal neural tissue
- adjacent spinal cord abnormal
- flaccid paralysis below lesion, incontinence, urinary tract dilatation
- surgical closure and hydrocephalus drainage
Mx eneurisis
- fluid restriction before bed and empty bladder
-star chart - eneurisis alarm
desmopressin
Mx eczema
- Mild = 1% hydrocortisone
- Mild - moderate = clobetasome 0.05%
- moderate to severe = monentason furouate
S+S dka
Dehydration
Kussmaoul
Smell ketones
lethargy, drowsiness
DKA biochemistry
- Glucose >11 Acidameia = <7.3 - Ketones >3 Mild = pH <7.3, 5% dehydration Mod = <7.2 and 7% dehydration Sev = <7.1 10% dehydration
IV Mx DKA
- 10ml/kg fluid bolus over 60 minutes
- Correct fluid deficit over 48hrs
- 0.9% NaCl with 20mmol KCL in every 500ml
- Insulin 0.05 or 0.1 units/kg/hr 1-2hrs after fluids
Rickets biochemical picture
- Reduced serum Ca and Phosphate
- Raised serum ALP
Esotropia
Inward positioned squint (affected eye towards nose)
Exotropia
- Outward positioned squint (affected eye towards ear)
Hypertropia
Upward moving affected eye
Hypotropia
Downward moving affected eye
causes of squint
- isiopathic
- hydrocephalus
- cerebral palsy
= space occupying lesions - trauma
Hirschberg’s test
- pen torch from 1m
- observe reflection of light source on cornea
- Deviation from centre = squint
Cover test
- Cover 1 eye and ask patient to focus on an object in front
- Move cover to opposite eye and watch movement of other eye
- If move inwards it has drifted outwards when covered = exotropia
- If moves outwards it has drifted inwards when covered = eso
Audiometry
- <3y tested for basic response to sound
- Older = specific tones and volumes
- Audiometry recorded on audiogram = identify conductive and sensorineural hearing loss
Audiogram
- Establish the minimum volume required for the patient to hear each frequency
- Air and bone conduction tested seperately
- Normal hearing = between 0 and 20 DB
Sensorineural hearing loss
- Air and bone more than 20 DB
Conductive hearing loss
- Bone - normal
- Air = greater than 20db
what hearing test for 6-18m
distraction
what hearing test for 3y+
pure tone audiomentry
what hearing test for 24+m
performance test
what hearing test for 6-30m
visual reinforcement
Mixed
- Air abd bone more than 20
- Difference of 15db between the 2
Mx ADHD
- Methylphenidate (ritalin)
- Dexamfetamine
- Atomoxetine
IM adrenaline doses >12y
- 500mcg IM (0.5ml)
Adrenaline age 6-12y
300mcg IM
adrenaline 6m-6y
- 150mcg
adrenaline <6m
100-150mcg
roseola infantum
- Herpes simplex 6
- after fever subsides
- SE = febrile seizures
Nephrotic complications
- Hypercholesterolaemia = cholesterol correlates inversely with serum albumin
- Thrombosis
- Infection
- Hypovolaemia
amblyopia
- Defective visual acuity
- Persists after correction of refractive error and removal of any pathology
<3m S+S UTI
- FTT
- Irritable, lethargy
- Fever, vomiting
- Poor feed
- <3m suspect UTI = refer = abx, sepsis screen, mc+s
> 3m S+S UTI
- Continence change
- Vomiting
- Frequency,dysuria
- Pain and loin tenderness
- Poor feed
when do you need a urine sample
- Fever >38
- S+S
- <3m
- Alternative site infection but stays unwell
Mx UTI
- > 3m pyelo = cef or coamox
- > 3m cystisis = triemth or nitro
- <3m = IV cef
Atypical UTI
- Non e coli
- increased creatinine
- Fail respond 48hrs
- mass felt
ix - MCUG
- DMSA scan
- USS
Impetigo
- Staph aureus
- Off school until dry and scabbed or 48hrs abx
- Topical 1% hydrogen peroxide 5 days if non-bullous or uncomplicated
- 2% fusidic acid or mupirocin for 5 days if hydro not tolerated or around eyes or ineffective
Turners heart issues is…
bicuspid aortic valve or coarctation aorta
coarctation of aorta findings
- crescendo decrescendo murmur in upper left sternal border
CF inheritance
Autosomal recessive
scarlet fever caused by
- strep pyogenes = GAS
- sanpapaer rash, strawberry tongue, circumoral pallor
- mx = phenoxymethylpenicillin
what are the live vaccines
- MMR
- BCG
- Chickenpox
- nasal flu
- rotavirus
when do children get MMR
- 1 year old
- 3yr and 4m
what is in the 6 in 1
- diptheria
- tetanus
- pertussis
- polio
- Hib
- hep b
when do children get Men B
- 16 weeks
- 1 year
Formula for weight if not given
(Age + 4) X 2
Maintenance fluids
- 1st 10kg = 100
- next 10kg = 50
- every other kg = 20
0.9% nacl + 5% glucose
deficit fluids
% X 10 X Kg
Bolus
- 10 mls/kg
0.9% NaCl
DKA shocked fluids
10ml/kg bolus saline over 15 minutes and dont subtract later
DKA not shocked
10ml/kg bolus saline over 30 minutes and do subtract later
fluid therapy DKA
{(deficit - unshocked bolus)/48} + maintenance hrly
- 0.9% saline and 20mmol K in every 500ml bag
NICE guidelines for suspect cerbral palsy
- not sitting by 8 months
- not walking by 18m
- early hand prefference before 1y
- toe walking
fraser guidelines
- cant be persuaded to inform parents
- understands advice or tx
- suffer if do not receive
- best interests
- continue sex without treatment
