Paediatrics Flashcards
What are the organisms causing newborn pneumonia?
Group B streptococcus from mother’s genital tract
Gram-neg enterococci
What organisms cause pneumonia in infants and young children?
Respiratory viruses (RSV in particular)
Streptococcus pneumonia
Haemophilus influenza
Bordetella pertussis
What organisms cause pneumonia in children over 5
Mycoplasma pneumoniae
Streptococcus pneumoniae
Chlamydia pneumoniae
What are the classic signs of consolidation?
Dullness on percussion
Decreased breath sounds
Bronchial breathing over the affected area
(these are often absent in young children)
What is the antibiotic of choice for pneumonia
Amoxicillin or erythromycin for over 5 year olds
Co-amoxiclav if complicated or unresponsive
What is the defect in cystic fibrosis
Defective protein in the cystic fibrosis transmembrane conductance regulator (CFTR) on chromosome 7
What is CFTR
A cyclic AMP-dependent chloride channel
What is the pathophysiology of CF?
Abnormal ion transport across epithelial cells
What are the other complications of CF apart from resp?
Meconium ileus
Blocks pancreatic ducts - pancreatic enzyme deficiency and malabsorption
Abnormal sweat gland function
What is the diagnostic test for CF
The sweat test - high chloride concentration
And gene testing
What is the most common causative organisms for peri orbital cellulitis
Staphylococcus Aureus
Group A strep
Haemophilus Influenzae
What should be excluded in diagnosis of a squint
Retinoblastoma - check red reflexes
Cataracts
After how many months old should a baby with a squint be referred to a specialist?
3 months - newborn babies normally have transient misalignments
What are the two types of squints?
Concomitant (usually due to a refractive error in one eye)
Paralytic (sinister causes such as a space-occupying lesion)
How to test for a squint with a pen-torch
Red reflex in both eyes at the same time - if not, there is a squint
Severe GORD is more common in children with what?
Cerebral palsy and neurodevelopment disorders
Preterm infants
Following surgery fro oesophageal atresia or diaphragmatic hernia
Complications of GORD
Failure to thrive due to severe vomiting
Oesophagitis
Recurrent pulmonary aspiration
Dystonic neck posturing
Apparent life-threatening events (ALTE)
How is failure to thrive defined on a growth chart?
Mild: a fall across 2 centile lines
Severe: a fall across 3 centime lines
Causes of failure to thrive
Inadequate intake
Inadequate retention
Malabsorption
Failure to utilise nutrients
Increased requirements
What is marasmus
Weight for height 3 standard deviations below the median and a wasted, wizened appearance
Skin-fold appearance and id-arm circumference markedly reduced
What is Kwashiorkor
Severe protein malnutrition with generalised oedema as well as severe wasting. Weight may not be reduced due to oedema
Can happen when infants are weaned from the breast quite late or if diet is high in starch
Features of Kwashiorkor
‘Flaky-paint’ skin rash with hyper keratosis and desquamation
Distended abdomen and enlarged liver
Angular stomatitis
Hair which is sparse and depigmented
Diarrhoea, hypothermia, bradycardia and hypotension
Low plasma albumin, potassium, glucose and magnesium
Management of severe acute malnutrition
Hypoglycaemia
Hypothermia
Dehydration (but avoid being overzealous with IV fluids as can lead to heart failure)
Electrolytes
Infection
Micronutrients
What is Hirschprung disease
The absence of myenteric plexuses of rectum and variable distance of colon. Causes a narrow, contracted segment
How does Hirschsprung disease present
Intestinal obstruction in the newborn period following delay in passing meconium. Or in later childhood - profound chronic constipation, abdominal distension and growth failure. Digital rectal exam can cause a release of stool and flatus
How does Meckel diverticulum present
Generally asymptomatic, but may present with bleeding, intussusception, volvulus or diverticulitis
What is biliary atresia
Destruction or absence of the extra hepatic biliary tree and intra-hepatic biliary ducts. Untreated, it leads to chronic liver failure and death.
What is the surgical management of biliary atresia?
Hepatoportoenterostomy (Kasai procedure) to bypass the fibrosed ducts
What are choledochal cysts?
Cystic dilatations of the extra hepatic biliary system
How do choledochal cysts present?
In infancy with cholestasis. In older age, present with abdominal pain, a palpable mass, jaundice or cholangitis.
How does neonatal hepatitis syndrome present?
Prolonged neonatal jaundice and hepatic inflammation
May have intrauterine growth restriction and hepatosplenomegaly at birth
How does neonatal hepatitis syndrome present?
Prolonged neonatal jaundice and hepatic inflammation
May have intrauterine growth restriction and hepatosplenomegaly at birth
What can cause neonatal hepatitis syndrome
Alpha1-antitrypsin deficiency
Galactosaenia
+ other causes
Causes of acute liver failure in children
Paracetamol overdose
non-A to G viral hepatitis
Metabolic conditions
How does acute liver failure in children present?
Jaundice
Encephalopathy
Coagulopathy
Hypoglycaemia
Electrolyte disturbance
Investigation findings in acute liver failure
Very high transaminases
Very abnormal coagulation
Elevated plasma ammonia
What are the complications of acute liver failure
Cerebral oedema
Haemorrhage from gastritis or coagulopathy
Sepsis
Pancreatitis
Management of acute liver failure
Maintain blood glucose - IV dextrose
Prevent sepsis - broad spectrum Abx and antifungals
Prevent haemorrhage - IV vit K, fresh frozen plasma or cryoprecipitate and H2-blocking drugs or PPIs
Treat cerebral oedema - fluid restrict and mannitol diuresis
LIVER TRANSPLANT
What medication is Reye syndrome linked to
Aspirin in under 12s
What is Reye syndrome
Acute non-inflammatory encephalopathy with micro vesicular fatty infiltration of the liver
When do you consider treatment for nocturnal enuresis
After 6 yrs old
What is the management for nocturnal enuresis
Star charts
Enuresis alarm
Sometimes desmopressin
Pre-renal causes of acute renal failure (most common in children)
Hypovolaemia (D&V, burns, sepsis, haemorrhage, nephrotic syndrome)
Circulatory failure
Renal causes of acute renal failure
Vascular (haemolytic uraemia syndrome, vasculitis, embolus, renal vein thrombosis)
Tubular (acute tubular necrosis, ischaemic, toxic, obstructive)
Glomerular (glomerulonephritis)
Interstitial (interstitial nephritis, pyelonephritis0
Post-renal causes of acute renal failure
Obstruction (congenital or acquired)
What is haemolytic uraemic syndrome?
Triad of:
Acute renal failure
Haemolytic anaemia
Thrombocytopaenia.
Often occurs after an infectious diarrhoea like Shigella
Causes of chronic renal failure in children
Structural malformations
Glomerulonephritis
Hereditary nephropathies
Systemic diseases
Features of chronic renal failure in children
Anorexia and lethargy
Polydipsia and polyuria
Failure to thrive/growth failure
Bony deformities from renal osteodystrophy (renal rickets)
Hypertension
Acute-on-chronic renal failure (precipitated by infection or dehydration)
Incidental finding of proteinuria
Unexplained normochromic, normocytic anaemia
What does acute nephritis lead to:
Decreased urine output and volume overload
Hypertension, which may cause seizures
Oedema, characteristically around the eyes
Haematuria and proteinuria
What can cause nephritic syndrome (acute nephritis)?
Post-streptococcal or post-infectious nephritis
Henoch-schönlein Purpura (or other types of vasculitis)
IgA nephropathy and mesangiocapillary glomerulonephritis
Anti-glomerular basement membrane disease (Goodpasture syndrome)
Familial (Alport syndrome. An X-linked recessive)
Signs and symptoms of Henoch-Schönlein purpura
Rash - buttocks, extensor surfaces of legs and arms, ankles
Joint pain and swelling
Abdo pain (haematemesis and melaena, intussusception)
Renal (microscopic/macroscopic haematuria, nephrotic syndrome)
What is hereditary angioedema
Rare autosomal dominant
No urticaria but subcutaneous swellings with abdominal pain.
Usually triggered by trauma
Can cause resp obstruction
Can be treated in the short term with fresh frozen plasma replacement
What is erythema nodosum
Tender nodules over the legs
Often also have a fever and arthralgia
Causes of erythema nodosum
Streptococcal infection
Primary TB
IBD
Drug reaction
Idiopathic
What is erythema multiforme
Target lesions with a central papule surrounded by an erythematous ring. Lesions may also be vesicular or bullous
Causes of erythema multiforme
HSV
What is the age range for febrile seizures?
6 months to 6 years old
Febrile seizures don’t normally increase risk of epilepsy. What types of febrile seizures do?
Focal, prolonged or repeated in the same illness. Need to rule out intracranial infection like meningitis or encephalitis
What is the age of onset for West syndrome (epileptic syndrome)
4-6 months
What is the age of onset for Lennox-Gastaut syndrome (epileptic syndrome)
1-3 years
What is the age of onset for childhood absence epilepsy?
4-12 years
What is the age of onset for benign epilepsy, with centrotemporal spikes (BECTS)
4-10 years
What is the age of onset for early-onset benign childhood occipital epilepsy?
1-14 years
What is the age of onset for juvenile myoclonic epilepsy
Adolescence-adulthood
What can absence seizures be induced by?
Hyperventilation
What is the prognosis for childhood absence seizures
95% remission in adolescence, 5-10% develop tonic-clonic seizures in adult life
What is the treatment for West syndrome (epileptic syndrome)
Corticosteroids or vigabatrin
What is the first line drug for focal seizures?
Lamotrigine
What two features of development are autistic children grossly delayed in?
Social skills
Communication skills
What is the limit age for sitting without support?
9 months (median is 6 months)
What is the limit age for pushing up on arms and holding head up
3 months (median is 6 weeks)
What is the median age fro cruising around furniture
9-10 months (limit is 13 months)
What are the median and limit ages for fixing and following?
6 weeks
3 months
What are the median and limit ages for reaching out for toys
4 months
6 months
What are the median and limit ages for transferring between hands?
7 months
9 months
What are the median and limit ages for pincer grip
10 months
12 months
What are the median and limit ages for polysyllabic babble
3-4 months
7 months
What are the median and limit ages for indiscriminate consonant babble (dada, mama)
7 months
10 months
What are the median and limit ages for saying 6 words with meaning
12 months
18 months
What is the limit age for joining words
2 years
What is the limit age for 3 word sentences
2 1/2 years
What are the median and limit ages for a responsive smile
6 weeks
8 weeks
What are the median and limit ages for symbolic play
18-24 months
2-2 1/2 years
What are the median and limit ages for interactive play
2.5-3 years
3-3.5 years
Features of fractures that are likely to be inflicted
Fracture in an immobile child
Rib fractures
Multiple fractures
Multiple fractures of different ages
Features of bruises that are likely to be inflicted
Shape of a hand or object
On neck like strangulation
Wrists or ankles that look like ligature marks
Bruises to the buttocks in a child less than 2 years or any age without a good explanation
Features of burns that are likely to be inflicted
Any burn in a child who is not mobile
A burn in the shape of an implement - cigarette, iron
A ‘glove or stocking’ burn consistent with forced immersion
Features of bites that are likely to be inflicted
Bruising in the shape of a bite thought unlikely to have been caused by a young child
What investigation should be done in patients you suspect physical abuse fractures?
Full radiographic skeletal survey with oblique views of the ribs if under 30 months of age (2.5 yrs old)
What medical conditions should you consider in suspected child abuse?
Coagulation disorders
Osteogenesis imperfecta
Scalds and cigarette burns - may be bullies impetigo or scalded skin syndrome
What investigations should be done in suspected brain injury
CT immediately followed by MRI
Skeletal survey
Expert ophthalmological examination to identify retinal haemorrhages
Coag screen
What chromosomal abnormality is present in Edward’s syndrome
Trisomy 18
What chromosomal abnormality is present in Patau’s syndrome
Trisomy 13
Clinical features of Edwards syndrome
Low birthweight
Prominent occiput
Small mouth and chin
Short sternum
Flexed, overlapping fingers
Rocker-bottom feet
Cardiac and renal malformations
Clinical features of Patau’s syndrome
Structural defect of brain
Scalp defects
Small eyes(microphthalmia) and other eye defects
Cleft lip and palate
Polydactyly
Cardiac and renal malformations
What is the prevalence of fragile X syndrome
1 in 4000
What causes fragile x syndrome
CGG trinucleotide repeat expansion
Normal is below 50 repeats
Pre-mutation is 55-199
Full mutation is 200 +
Do female carriers of fragile x syndrome experience any symptoms
50% do and have mild to moderate learning difficulties
What are some of the characteristic facies of someone with fragile X
Protruding/everted ears
Long face
Prominent mandible
Broad forehead
What are the non-facial features of fragile X
Macrocephaly
Macro-orchidism
Mitral valve prolapse, joint laxity, scoliosis, autism, hyperactivity
What are the clinical features of Noonan syndrome
Occasional mild learning difficulties
Short webbed neck with trident hair line
Pectus excavatum
Short stature
Congenital heart disease (especially pulmonary stenosis, atrial septal defect)
Characteristic facies
What is the inheritance pattern for Noonan syndrome
Autosomal dominant
What are the clinical features of Williams syndrome
Short stature
Transient neonatal hypercalcaemia (occasionally)
Congenital heart disease (supravalvular aortic stenosis)
Mild to moderate learning difficulties
Characteristic facies
Signs of respiratory distress in a neonate
Tachypnoea (>60 breaths/min)
Laboured breathing, with chest wall recession (particularly sternal and subcostal indrawing) and nasal flaring
Expiratory grunting
Cyanosis if severe
Is meconium passed more frequently the more pre term or the more term
The greater the gestational age, affecting 20-25% of deliveries by 42 weeks