Paeds additional Flashcards
what is Hirschsprung’s disease
aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses.
condition associated with hirschsprung’s disease
- downs syndrome (MC)
- neurofibromatosis
- Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)
- Multiple endocrine neoplasia type II
presentation of hirschsprung’s disease
- Delay in passing meconium (more than 24 hours)
- Chronic constipation since birth
- Abdominal pain and distention
- Vomiting
- Poor weight gain + failure to thrive
Mx of Hirschsprung’s disease
- Ix= AXR and rectal biopsy
- initially: rectal washouts/bowel irrigation, fluid resus, IV abx
- definitive management: surgical removal of affected segment of the colon
Features of innocent murmurs
ALL S
- Soft
- Short
- Systolic
- Symptomless
- Situation dependent (the murmur gets quieter with standing, or only appears when the child is unwell or feverish)
pansystolic murmurs
- Mitral regurgitation, heard loudest in the mitral area
- Tricuspid regurgitation, heard loudest in the tricuspid area
- Ventricular septal defect, heard loudest at the left lower sternal border
causes of ejection systolic murmurs
- Aortic stenosis
- Pulmonary stenosis
- Hypertrophic obstructive cardiomyopathy
Heart defects that can cause a right-to-left shunt, and therefore cyanotic heart disease, are:
- ASD
- VSD
- PDA
- Transposition of the great arteries
Causes of anaemia in infancy
Physiologic anaemia = MC
The other causes of anaemia in infants are:
- Anaemia of prematurity
- Blood loss
- Haemolysis
- Twin-twin transfusion
Haemolysis is a common cause of anaemia in infancy. There are a number of causes of haemolysis in a neonate:
- Haemolytic disease of the newborn (ABO or rhesus incompatibility)
- Hereditary spherocytosis
- G6PD deficiency
what is physiological anaemia of infancy
normal dip in haemoglobin around 6-9 weeks of age in healthy term babies
causes of anaemia of prematurity
- Less time in utero receiving iron from the mother
- RBC creation cannot keep up with rapid growth in the first few weeks
- Reduced erythropoietin levels
- Blood tests remove a significant portion of their circulating volume
test to check for immune haemolytic anaemia
direct Coombs test
causes of anaemia in older children
- iron deficiency
- blood loss
Rarer causes
- sickle cell
- Thalassaemia
- Leukaemia
- Hereditary spherocytosis
- Hereditary eliptocytosis
- Sideroblastic anaemia
Symptoms specific to iron deficiency anaemia:
- Pica = dietary cravings for abnormal things such as dirt
- Hair loss
Sx of anaphylaxis
- Urticaria, itching, Angio-oedema, abdo pain, swelling around lips and eyes
- SOB
- Wheeze
- Stridor
- Tachycardia
- Lightheadedness
- Collapse
how to confirm anaphylaxis
mast cell tryptase within 6hrs
signs and sx of appendicitis
- centro abdo pain then RIF pain
- McBurney’s point (1/3 ASIS to umbilicus)
- N&V
- fever
- guarding and rebound tenderness
- Rovsing’s sign
mx of acute asthma
- oxygen <94%
- SABA (nebulised mod-severe, spacer mild-mod)
- nebulised ipratropium bromide
- oral prednisolone, IV if unable to swallow
- oral LTRA
- nebulised magnesium sulphate for severe asthma
Can consider
7.IV salbutamol
8. IV aminophylline
9. IV magnesIum sulphate
CALL SENIOR
How to diagnose asthma
> 5 y/o spirometry
FeNO
Peak Flow
Direct bronchial challenge test
steroids for eczema (mild, moderate, potent)
- Mild: Hydrocortisone 0.5%, 1% and 2.5%
- Moderate: Eumovate (clobetasone butyrate 0.05%)
- Potent: Betnovate (betamethasone 0.1%)
- Very potent: Dermovate (clobetasol propionate 0.05%)
most common organism in bacterail infection of eczema
staph aureus
what is eczema herpeticum caused by
herpes simplex virus (HSV) or varicella zoster virus (VZV)
HSV1 most common
presentation of eczema herpeticum
widespread, painful, vesicular rash
+ systemic symptoms such as fever, lethargy, irritability and reduced oral intake.
There will usually be lymphadenopathy
what is biliary atresia
section of the bile duct is either narrowed or absent –> cholestasis –> accumulation of conjugated bilirubin
presentation of biliary atresia
- jaundice due to high conjugated bilirubin levels shortly after birth lasting >14 days post birth
Ix for biliary atresia
conjugated and unconjugated bilirubin (can conj but just can’t excrete it)
what is bronchiolitis caused by
Respiratory syncytial virus (RSV)
presentation of bronchiolitis
- <1 yr, usually under 6 months
- particularly in ex-premature babies with chronic lung disease.
- Coryzal symptoms: VRUTI
- Signs of respiratory distress
- Dyspnoea
- Tachypnoea
- Poor feeding
- Mild fever (under 39ºC)
- Apnoeas
- Wheeze and crackles
signs of respiratory distress
- tachypnoea
- head bobbing
- nasal flaring
- use of accessory muscles
- intercostal and subcostal recessions
- tracheal tugging
- cyanosis
- abnormal airway noises
difference between wheeze, grunting and stridor
- Wheezing= whistling sound caused by narrowed airways, during expiration
- Grunting= exhaling with the glottis partially closed to increase PEEP
- Stridor= high pitched inspiratory noise caused by obstruction of the upper airway e.g. croup
progression of bronchiolitis
coryzal sx day 1-2
worst sx day 3-4
then improve, lasts 7-10 days
when to admit with bronchiolitis
- < 3months
- 50 – 75% reduced feed
- Clinical dehydration
- RR > 70
- O2 < 92%
- Moderate to severe respiratory distress
- Apnoeas
- Parents not confident in their ability to manage at home
prevention of bronchiolitis
Pavilizumab monthly injection
Causes of cerebral palsy
Antenatal:
- Maternal infections
- Trauma during pregnancy
Perinatal:
- Birth asphyxia
- Pre-term birth
Postnatal:
- Meningitis
- Severe neonatal jaundice
- Head injury
which parts of the brain are affected by different types of Cerebral palsy
Spastic- UMN
Dyskinetic- Basal Ganglia and substatntia nigra
Ataxis- Cerebellum
signs and sx of cerebral palsy
- Failure to meet milestones
- Increased or decreased tone, generally or in specific limbs
- Hand preference < 18 months = KEY
- Problems with coordination, speech or walking
- Feeding or swallowing problems
- Learning difficulties
complications of cerebral palsy
- Learning disability
- Epilepsy
- Kyphoscoliosis
- Muscle contractures
- Hearing and visual impairment
- Gastro-oesophageal reflux
Causes of constipation
- Functional
- Cystic fibrosis
- Hirschsprung’s disease
- Hypothyroidism
- Spinal cord lesions
- Sexual abuse
- Intestinal obstruction
- Anal stenosis
- Cows milk intolerance
age group for croup
6months- 2 years
cause of croup
parainfluenza virus
sx of CF
- meconium ileus
- failure to thrive
- chronic cough
- thick sputum
- recurrent resp infections
- steatorrhoea
- child salty when parent kisses
- abdo pain and bloating
- finger clubbing
- nasal polyps
how to diagnose CF
- Newborn blood spot testing
- The sweat test is the gold standard. Chloride conc >60mmol/L
- Genetic testing for CFTR gene
key colonisers in CF
Staphylococcus aureus- MC
Haemophilus influenza
Klebsiella pneumoniae
Escherichia coli
Burkhodheria cepacia
Pseudomonas aeruginosa- important
colonisers that exempt pt with CF from lung transplant
- burkholderia cepacia
- ?mycobacteria abscessus
conditions screened for in CF
- diabetes
- osteoporosis
- vitamin D deficiency
- liver failure
Mx of CF
- CFTR modulators (Kaftrio)
- chest physio
- vaccines (pneumo, influ, varicella)
- Nebulised DNase
- Creon
- high calorie diet
- prophylactic fluclox
- lung transplant
complications of Down syndrome
- Learning disability
- Recurrent otitis media
- Deafness. Eustachian tube abnormality–> glue ear and conductive hearing loss.
- Visual problems such myopia, strabismus and cataracts
- Hypothyroidism
- Cardiac defects
- Atlantoaxial instability
- Leukaemia
- Dementia: Alzheimer’s
tests for Downs syndrome
- combined test @ 11-14 weeks (high NT, high b-HCG, low PAPPA
- triple test @ 14-20 weeks (high b-HCG, low AFP, low serum oestriol)
- quadruple test @ 14-20 weeks (+ high inhibin
tests if high risk of Downs syndrome
- CVS
- amniocentesis
- NIPT
cause of epiglottitis
haemophilus influenza type B
presentation of epiglottitis
- Sore throat and stridor
-cDrooling - Tripod position
- High fever
- Difficulty or painful swallowing
- Muffled voice
- Scared and quiet child
- Septic and unwell appearance
Mx of epiglottitis
- secure the airway
- leave child alone
- IV abx (e.g. ceftriaxone)
- dexamethasone
what is Henoch Schonlein Purpura
- IgA vasculitis
- purple rash on bottom and legs
- IgA deposits in the blood vessels
- affects skin and GI tract
features of Henoch Schonlein Purpura
- Purpura (100%),
- Joint pain (75%),
- Abdominal pain (50%)
- Renal involvement (50%)
causes of hyposplenism
- splenectomy
- sickle-cell
- coeliac disease, dermatitis herpetiformis
- Graves’ disease
- systemic lupus erythematosus
- amyloid
- splenic sequestration crisis can lead to splenic infarction, leading to hyposplenism
features of hyposplenism
- Howell-Jolly bodies
- siderocytes
what is juvenile idiopathic arthritis (JIA)
arthritis without any other cause, lasting more than 6 weeks in a patient under the age of 16
subtypes of JIA
- Systemic JIA
- Polyarticular JIA
- Oligoarticular JIA
- Enthesitis related arthritis
- Juvenile psoriatic arthritis
systemic JIA
- Still’s disease
- Subtle salmon-pink rash
- High swinging fevers, weight loss
- Lymphadenopathy, splenomegaly
- Joint inflammation and pain
complication of systemic JIA
macrophage activation syndrome (MAS) =
- severe activation of the immune system + massive inflammatory response.
- acutely unwell child + DIC, anaemia, thrombocytopenia, bleeding and a non-blanching rash
polyarticular JIA
- 5 joints or more
- symmetrical
- mild fever, anaemia and reduced growth
- equivalent to RA in adults
oligoarticular JIA
- 4 joints or less
- affects larger joints, usually monoarthritis
- <6 y/o girls
- anterior uveitis
- ANA positive
enthesitis related arthritis
- male >6 y/o
- paediatric version of the seronegative spondyloarthropathy
- HLA B27 gene
- psoriasis
- IBD
- anterior uveitis
juvenile psoriatic arthritis
- seronegative inflammatory arthritis associated with psoriasis
- symmetrical affecting small joints or asymetrical large joints
- nail pitting
- onycholysis
- dactylitis
- enethesitis
causes of intestinal obstruction
- Meconium ileus
- Hirschsprung’s disease
- Oesophageal atresia
- Duodenal atresia
- Intussusception
- Imperforate anus
- Malrotation of the intestines with a volvulus
- Strangulated hernia
Mx of intestinal obstruction
- NBM
- BG tube drip and suck
- IV fluids
- treat cause
what is intussusception
bowel invaginates” or “telescopes” into itself
6months-2y/o
M>F
conditions associated with intussusception
- Concurrent viral illness
- Henoch-Schonlein purpura
- Cystic fibrosis
- Intestinal polyps
- Meckel diverticulum
presentation of intussusception
- Severe, colicky abdominal pain
- Pale, lethargic and unwell child
- “Redcurrant jelly stool”: late sign
- RUQ sausage shaped mass
- Vomiting
- Intestinal obstruction
mx of intussusception
- air insufflation under radiological control (1st line)
- therapeutic enema
- surgical reduction
- resection if bowel perforates
complications of intussusception
- obstruction
- gangrenous bowel
- perforation
- death
what is kawasaki disease
- systemic, medium-sized vessel vasculitis
- <5y/o
- no cause
- complication = coronary artery aneurysm
presentation of kawasaki disease
CRASH AND BURN
C- conjunctivitis
R- rash
A- lymphadenopathy
S- strawberry tongue
H- hand and feet swelling
Burn- >5days fever
mx kawasaki disease
- High dose aspirin to reduce the risk of thrombosis
- IVIG to reduce the risk of coronary artery aneurysms
- Echocardiograms for aneurysm
side effect of aspirin in children
Reye’s syndrome
what is Acute lymphoblastic leukaemia
- most common leukaemia in children
- 2-5 y/o
- T and B cell subtypes
Features of acute lymphoblastic leukaemia
- anaemia
- neutropaenia: frequent or severe infections
- thrombocytopenia: easy bruising, petechiae
- bone pain
- splenomegaly
- hepatomegaly
- fever in up to 50% of new cases
- testicular swelling
Mx of acute lymphoblastic leukaemia
chemo
2yr girls and 3yrs boys bc of testicles
what is impetigo
- superficial bacterial skin infection, usually caused by the staphylococcus aureus bacteria
- golden crust lesions
- contagious, keep off school
- bullous or non bullous
Non-Bullous impetigo
- nose or mouth
- golden crust
- hydrogen peroxide 1% 1st line then fusidic acid
- oral flucloxacillin for wide spread
Bullous impetigo
- fluid filled vesicles
- burst then golden crust
- in neonates to under 2 years
- flucloxacillin
Sx of measles
10-12 days after exposure
- Koplik spots
- conjunctivitis
- fever + coryzal sx
- rash starting on face behind ears then spread to the rest of the body
Mx of measles
self resolving
keep off school until 4 days post sx resolve
complication of measles
- Pneumonia
- Diarrhoea and dehydration
- Encephalitis and Meningitis
- Otitis media and vision loss
- Death
features of scarlett fever
- assoc w group A streptococcus infection
- sandpaper skin starting on trunk and spreading
- red cheeks
- strawberry tongue
features of rubella
- sx 2 weeks post exposure
- highly contagious, keep off school for at least 5 days after rash appears
- erythematous macular rash
- rash starts on face and spreads, last 3-5 days
- lymphadenopathy
- self-limiting
Congenital rubella syndrome
triad of deafness, blindness and congenital heart disease.
Parvovirus B19
- slapped cheek syndrome
- self-limiting
- infectious before rash appears
roseola infantum
- HHV6 and HHV7
- high fever and rash
- self-limiting
Features of mumps
- parotid swelling
- fever, headache etc.
- self limiting
what is muscular dystrophy
gradual weakening and wasting of muscles
what is pyloric stenosis
Hypertrophy and therefore narrowing of the pylorus.
This prevents food traveling from the stomach to the duodenum as normal.
Features of pyloric stenosis
- projectile vomiting
- forceful peristalsis
- hungry baby
- failure to thrive
- large olive mass in abdomen
- hypocholoric metabolic alkalosis
Mx of pyloric stenosis
- diagnose via USS
- laparoscopic pyloromyotomy (Ramstedt operation) widening canal
Causes of acute urticaria
triggered by something that stimulates the mast cells to release histamine. This may be:
- Allergies to food, medications or animals
- Contact with chemicals, latex or stinging nettles
- Medications
- Viral infections
- Insect bites
- Dermatographism (rubbing of the skin)
Chickenpox during pregnancy
- <28 weeks can cause congenital varicella syndrome
- at delivery can cause severe neonatal infection. Mx by varicella zoster immunoglobulins and aciclovir
whooping cough
- Bordetella pertussis
- inspiratory whoop
- nasal swab diagnosis
- self limiting
- azithromycin in first 21days
- prophylactic abx for vulnerable contacts
- 100 day cough
perthes
- avascular necrosis of femoral head
- 4-12yrs usually 5-8yrs
- pain in hip/groin, limp, reduced ROM
- no hx of trauma
- mx <6 good progonsis rest, crutches, traction, physio. surgery if doesn’t work >6
what is necrotising enterocolitis
- affects premature neonates
- part of the bowel becomes necrotic
- emergencyp
presentation of necrotising enterocolitis
- Intolerance to feeds
- Vomiting with green bile
- Generally unwell
- Distended, tender abdomen
- Absent bowel sounds
- Blood in stools
- When perforation occurs there will be peritonitis and shock
Mx of necrotising enterocolitis
- nil by mouth
- IV fluids
- total parenteral nutrition
- broad spectrum antibiotics
- surgery
caput succedaneum
- fluid collection with poorly defined margins caused by the pressure of the presenting part of the scalp against the dilating cervix
- crosses suture lines
- present at birth
- self resolving
caput cephalohaematoma
- develops several hours after delivery and is due to bleeding between the periosteum and skull
- parietal region
- 3 months to resolve
- doesn’t cross suture lines
what is transient synovitis
- Acute hip pain following a recent viral infection
- 5-8 y/o
- limp/refusal to weight bear
- groin or hip pain
- a low-grade fever is present in a minority of patients
- self-limiting
what organism is threadworms
Enterobius vermicularis
fragile X features
- males
- learning difficulties
- large low set ears, long thin face, high arched palate
- macroorchidism
- hypotonia
- autism is more common
- mitral valve prolapse
Features of patau’s syndrome
- trisomy 13
- Microcephalic, small eyes
- Cleft lip/palate
- Polydactyly
- Scalp lesions
Features of Edward’s syndrome
- trisomy 18
- Micrognathia
- Low-set ears
- Rocker bottom feet
- Overlapping of fingers
features of Noonan syndrome
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
Features of Pierre-Robin syndrome
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
features of prader willi syndrome
Hypotonia
Hypogonadism
Obesity
features of william’s syndrome
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
features of Crit du chat syndrome
Characteristic cry (hence the name) due to larynx and neurological problems
Feeding difficulties and poor weight gain
Learning difficulties
Microcephaly and micrognathism
Hypertelorism
where to palpate for pulse in paeds BLS
<1 = femoral or brachial
>1 = femoral or carotoid
salter harris fracture classification
1- (straight) through physis
2. (above) through physis and metaphysis
3. (low) physis and epihysis
(through) physis, epiphysis, metaphysis
4.(er crush) fracture
tetralogy of fallot features
PROVE
- Pulmonary stenosis
- Right ventricular hypertrophy
- Overriding aorta
- VEntricular septal defect
sx of tetralogy of fallot
cyanotic spells, along with poor feeding, agitation and low birth weight