Paeds Passmed Flashcards
What is the treatment for threadworm?
Single dose mebendazole for whole family (>6 months)
How do you check pulse on infants vs children?
Infants: Brachial + femoral
Children: Femoral
What valvular abnormalities are present in turners syndrome? And how do they present?
- Bicuspid aortic valve (ejection systolic upper right sternal border which radiates to the carotids
- Coarctation of the aorta (Systolic murmur that is loudest in the back below the left scapula)
- aortic root dilatation
What is turners syndrome?
Only affects females. Missing/partially missing x-linked chromosome. XO karyotype
How does turners present?
- Short stature
- Webbed neck + low set ears
- primary amenorrhea + poorly developed secondary characteristics
- High arched palate
- Raised FSH/LH + low oestradiol -> POF
- horseshow kidney
What chromosome is affected in pataus?
Trisomy 13. Not inheritied - nondisjunction or translocation
How does pataus present?
- microcephalic, small eyes
- cleft lip/palate
- polydactyly
- Scalp lesions
- Incompatible with life
What chromosome is affected in Edwards?
Trisomy 18
How does Edwards present?
- IUGR
- Micerognathia
- Overlapping fingers
- Rocker bottom feet
- Incompatible with life
- choroid plexus cysts
How does fragile x present?
- Learning difficulties/autism (lack of protein that causes brain to develop)
- Macrocephaly
- Long face
- Large ears
- Macro-orchidism
- mitral valve prolapse
3 Ms, 3 Ls
Trinucleotide disorder
How does Noonans present?
- Webbed neck
- Pectus excavatum
- short stature
- Pulmonary stenosis
- Wide spaced eyes + deep philtrum
What causes kleinfelters syndrome?
Having an addition X chromosome (not directly inherited). Only affects men.
How does Klinefelter’s present?
- Often asymptomatic till puberty
- infertility (small testes + primary testicular insufficiency)
- Taller, curved pinky, flat feet
- symptoms of low testosterone -> gynecomastia, decreased body hair
How is Klinefelter’s treated pharmacologically?
TRT
What chromosome is affected in prader-willi syndrome?
Loss of function of chromosome 15
How does prader-willi present?
- Excessive appetite/eating
- Hypotonia
- Hypogonadism
- Restricted growth
What chromosome is affected in williams syndrome?
Partial loss of chromosome 7
How does Willams present?
- Supravalvular aortic stenosis (not cyanotic)
- Intellectutal disability
- Friendly
- Broad forehead, strabimus, elfin facies
What are the 3 symptoms of shaken baby syndrome?
- Retinal haemorrhage
- Subdural haemorrhage
- Encephalopathy
Why should aspirin never be given to children?
Reyes syndrome
How does perthes disease present?
- Hip pain
- Limping
- Reduced range of motion
- Widening of joint spaces
- Leg shortening
What does a jittery and hypotonic newborn indicate?
Neonatal hypoglycaemia
What is the gold standard test for hirsphrungs?
Rectal biospy - lack of ganglionic nerve cells
What congential heart condtiion is associated with downs?
- Tetralogy of Fallot (overriding aorta, RVH, VSD, pulmonary stenosis -> Ejection systolic murmur )
- ASD: a systolic murmur is heard at
the upper left sternal edge which radiates to the back. (most common)
What GI condition is secondary to cystic fibrosis?
Meconium ileus - small bowel obstruction caused by thickened meconium
Symptoms of ALL?
- Easy bruising
- Hepatosplenomegaly
- SOB and systolic murmur
What is raised in biliary atresia?
- Conjugated billirubin - as it is normally released in bile
- Bile acids and aminotransferases are also typically raised, but cannot be used to differentiate between other causes of neonatal cholestasis and biliary atresia.
What is the treatment for biliary atresia ?
Kasai procedure
What makes a murmur innocent?
Soft, systolic, short, Symptomless, Standing/Sitting (vary with position)
Side effect of maternal anti-epileptic use?
Cleft lip
Where does inflammation occur in osgood-schatter
tibial tuberosity
Most common cause of primary amenorrhea?
Androgen insensitivity syndrome
How do you differentiate perthes vs transient synovitis?
Transient synovitis is usually following another infection and perthes more common in boys
Most common sign of neonatal sepsis?
Resp distress/grunting
What condition usually precedes ITP?
Viral infection
What is the treatment for croup?
- single dose of dexamethasone
- inhaled racemic adrenaline
What are the cyanotic congenital heart diseases?
- Tetralogy of fallot (a few months)
- Transposition of the great arteries (right after birth + diabetic mother)
- tricuspid atresia
- Total anomalous pulmonary venous return
- Truncus arteriosus
What is given in cyanotic CHD?
Prostaglandin E1 - to prevent closure of PDA to allow mixing of oxygenated and deoxygenated blood until surgical correction
Child with billous vomit, diarrhoea, reduced feeding, PROM?
Necrotising entercolitis - xray ( Dilated Bowel Loops, Bowel wall oedema, Pneumotitis Intestinalis: gas within the wall of the intestine)
What conditions is cystic fibrosis associated with?
- Diabetes
- Downs
- meconium illeus
Jaundice in the first 24 hours is always…
pathological - blood film analysis
Causes of jaundice in <24 hours?
- rhesus haemolytic disease
- positive coombs test
- normocytic anaemia with reticulocytosis and bilirubinaemia.
- nucleated red blood cells - ABO haemolytic disease
- hereditary spherocytosis
- negative coombs test - glucose-6-phosphodehydrogenase
What antibiotic is used for whooping cough?
Clarithomycin
How does hirshprungs present/diagnosed/treated?
- Delayed meconium passing (48 hours)
- Absence of ganglion cells from myenteric and submucosal plexuses
- associated with downs and MEN IIa
Diagnosis: Full-thickness rectal biopsy for diagnosis
Treatment: rectal washouts initially, after that an anorectal pull-through procedure/swenson
How does pyloric stenosis present/diagnosed/treated?
1 Projectile non bile stained vomiting at 4-6 weeks of life
2. hypochloraemic, hypokalaemic alkalosis due to vomiting
Diagnosis: test feed or USS
Treatment: Ramstedt pyloromyotomy (open or laparoscopic)
How does mesenteric adenitis present?
- Right lower quadrant pain secondary to an inflammatory condition of mesenteric lymph nodes (URTI)
Treatment: conservative management
How does intussusception present/diagnosed/treated?
- Telescoping bowel, proximal to or at the level of, ileocaecal valve
- 6-9 months of age
- Colicky pain, diarrhoea and vomiting, sausage-shaped mass, red jelly stool.
Diagnosis: ultrasound (target sign)
Treatment: reduction with air insufflation
How does intestinal malrotation present/diagnosed/treated?
- High caecum at the midline
- Feature in exomphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia
- May be complicated by the development of volvulus, an infant with volvulus may have bile stained vomiting
Diagnosis: upper GI contrast study and USS
Treatment: laparotomy, if volvulus is present (or at high risk of occurring then a Ladd’s procedure is performed (includes division of Ladd bands and widening of the base of the mesentery)
How does oesophageal atresia present?
- Associated with tracheo-oesophageal fistula and polyhydramnios
- May present with choking and cyanotic spells following aspiration
- VACTERL associations (Vertebral, anal atresia, cardiac defects, traceal anomalies, esophagel atresia, renal and radial thumb, limbs)
How does meconium ileus present/diagnosed/treated?
- Usually delayed passage of meconium and abdominal distension
- associated with cystic fibrosis
Diagnosis: X-Rays will not show a fluid level as the meconium is viscid, PR contrast studies may dislodge meconium plugs and be therapeutic
Treatment: Infants who do not respond to PR contrast and NG N-acetyl cysteine will require surgery to remove the plugs
How does biliary atresia present/treated?
- Jaundice > 14 days
- Increased conjugated bilirubin
Treatment: Urgent Kasai procedure (connection between liver and small intestine for bile drainage)
How does necrotising enterocolitis present/diagnosed/treatment?
- Prematurity is the main risk factor
- abdominal distension and passage of bloody stools
- billous vomit, diarrhoea, reduced feeding, PROM
- Increased risk when empirical antibiotics are given to infants beyond 5 days
Diagnosis: x-Rays may show pneumatosis intestinalis and evidence of free air (GAS CYSTS) or dilated bowel loops
Treatment: total gut rest and TPN, babies with perforations will require laparotomy + broad spectrum antibiotics
How does Meckel’s diverticulum present/diagnosed?
- abdominal pain mimicking appendicitis
- painless rectal bleeding (most common cause of painless GI bleed requiring transfusion in kids 1-2)
- intestinal obstruction: secondary to an omphalomesenteric band (most commonly), volvulus and intussusception
Investigation: Meckel/technetium scan
Treatment: wedge excision (if symptomatic)
How is kawasaki disease treated?
- High dose aspirin
- IV immunoglobulin
- Echo (cornoary artery aneurysm)
What triggers DIC?
major trauma, sepsis, severe obstetric disorders and malignancy
What kind of vaccine is the rotavirus? and when is it administered?
Oral, live, attenuated vaccine
Given at 2 and 3 months
Risk of intussusception if given at wrong time
What is the side effect of methylphenidate? and what is given if not tolerated
(dopamine/norepinephrine reuptake inhibitor)
cardiotoxic. Perform a baseline ECG before starting treatment
stunted growth - monitor every 6 months
lisdexamfetamine/dexafetamine
How does pierre-robin present?
- Micrognathia
- Posterior displacement of the tongue (may result in upper airway obstruction)
3.Cleft palate
When are unilateral undescended testes concerning?
> 3 months
bilateral: 24 hours
increased risk of testicular torsion, infertility, cancer
What are the NICE red flag symptoms?
- Moderate or severe chest wall recession
- Does not wake if roused
- Reduced skin turgor
- Mottled or blue appearance
- Grunting
- appears unwell to doctor
What is the treatment for PDA?
Indomethacin or ibuprofen
Which condition is associated with pulmonary hypoplasia?
Congenital diaphragmatic hernia
What is used to prevent RSV?
Palivizumab: monoclonal antibody
When should a child with fever be admitted?
< 3 months + > 38 degrees ALWAYS admit
How is meningitis treated?
> 3 months: IV cefritaxime/one + dexamethasone
< 3 months: IV cefritaxime + amoxicillin (listeria) -> no steroids
Add vancomycin if recent travel outside UK
How does transposition of the great arteries present?
no murmur but typically a loud single S2 is audible and a prominent right ventricular impulse is palpable on examination
What is the most common cause of stridor in infants?
Laryngomalacia - floopy epiglottis that fols in wards and worse when eating
When should an urgent referral be made with an acute limp?
< 3 years
What investigation do all breech babies require?
USS at 6 weeks
What is the recommended compression: ventilation ratio for the newborn?
3:1
How does a slipped capital femoral epiphysis present? diagnosed? treated?
- loss of internal rotation of the leg in flexion
- hip, groin, medial thigh or knee pain
Frog pose hip x-ray
Treatment: internal fixation: typically a single cannulated screw placed in the center of the epiphysis
What is the causative organism for roseola infantum?
human herpes virus 6
What is the causative organism for slapped cheek syndrome?
Parovirus B19
What is the causative organism for hand, foot and mouth disease?
Cosackie A16
How does rubella present?
1..pink maculopapular, initially on face before spreading to whole body, usually fades by the 3-5 day
2. Lymphadenopathy: suboccipital and postauricular
How is GORD treated?
2 week alginate -> 4 week PPI or H2 receptor antagonist (ranitidine) -< Nissan fundoplication
How does osgood-schatter present?
- Unilateral (but may be bilateral in up to 30% of people).
- Gradual in onset and initially mild and intermittent, but may progress to become severe and continuous.
- Relieved by rest and made worse by kneeling and activity, such as running or jumping.
Most common causative organism of acute epiglottis.
Haemophilus influenzae type B
How do you differentiate between seizure and psudoseizure?
Seizure - increased prolactin
Which vaccine would you offer for uni students?
Men ACWY
How do you differentiate between Kawasaki and scarlet fever?
Kawasaki has fever >5 days
What is one of the few indications for aspirin in children?
Kawasaki disease
Difference in metabolic disorders with vomiting vs diarrhoea?
Vomiting: hypochloremic, hypokalaemia metabolic alkalosis
Diarrhoea: metabolic acidosis
How is CMPA treated? and what is the prognosis?
- eHF
- AAF
IgE mediated resolves by 5
Non-IgE mediated resolves by 3
How are undescended testes managed?
Unilateral: wait till 3 months
Bilateral: 24 urgent refferal -> karyotping
What is Perthes disease?
avascular necrosis of femoral head - 4-8 years
What is exomphalos
the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum -> may have raised AFP
How is exomphalos managed?
c-section and staged repair
What is a late complication of downs syndrome?
- Alzheimers - chromosome 21 produces amyloid plaques
- ALL
- Hypothryodiism
- subfertility
How do you differentiate between caput succedaneum and cephalohematoma?
Caput crosses suture lines and present at birth
What cranial injuiry most commonly causes seizures after birth?
intracranial haemorrhage
1. Subarachnoid haemorrhages are common and may cause irritability and even convulsions over the first 2 days of life.
2. Subdural can following the use of forceps.
3. Intraventricular haemorrhage mostly affects pre-term infants and can be diagnosed by ultrasound examinations.
What complication of chicken pox causes systemic symptoms with peeling grey wound?
Group a step -> necrotising fasciitis
id not systemic symptoms -> cellulitis
How is vit K given to newborns?
one-off oral/IM dose to prevent HDN
How are immunisations given to preterm babies?
Acorrding to chronological age, not gestations
if <28 weeks give in hospital
How is symptomatic neonatal hypoglycaemia treated?
admit to nepnatual unit and 10% iV dextrose
What cardiac abnormality is seen with duchenne muscular dystrophy?
Dilated cardiomyopathy
How does JIA present?
- pyrexia
- salmon-pink rash
- lymphadenopathy
- arthritis
- Chronic anterior uveitis (There is a national screening programme for all children with JIA to have their eyes screened on a 3 monthly basis)
- anorexia and weight loss
How is JIA diagnosed?
- ANA may be positive, especially in oligoarticular JIA
rheumatoid factor is usually negative - <16 years and >6 months
How does cystic fibrosis present?
- Meconium ileus (neonatal)
- recurrant chest infections
- malabsoption/failure to thrive
- nasal polyps
- delayed puberty
- rectal prolapse
- diabetes mellitus
What are the hearing tests for each age group?
- Newborn - otoacoustic emmision test + auditory brainstem response test
- 6-9 months - distraction test
- 1.5-2.5 years - Recognition of familiar objects
- school age - pure tone audiometry
How does PDA present?
- left subclavicular thrill
- continuous ‘machinery’ murmur
- large volume, bounding, collapsing pulse
- wide pulse pressure
- heaving apex beat
What is TGA and how does it present?
Aorta connected to R ventricle instead of left - pumps deoxygenated blood to body
Pulmonary artery connected to L ventricel instead of R - pumps ocygenated blood to lungs
- cyanosis
- tachypnoea
- loud single S2
- prominent right ventricular impulse
- ‘egg-on-side’ appearance on chest x-ray
How do you manage children with unexplained mass (+ painless haematuria)?
48 hour paediatric referral - could be wilms tumours (nephroblastoma)
What are the complications of measles?
otitis media: the most common complication
pneumonia: the most common cause of death
encephalitis: typically occurs 1-2 weeks following the onset of the illness)
subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness
febrile convulsions
keratoconjunctivitis, corneal ulceration
diarrhoea
increased incidence of appendicitis
myocarditis
What is the treatment for cerebral palsy?
- oral diazepam
- oral and intrathecal baclofen
- botulinum toxin type A
How is cerebral palsy classifed?
- spastic (70%)
- subtypes include hemiplegia, diplegia or quadriplegia
- increased tone resulting from damage to upper motor neurons (pyramidal tracts/corticospinal0 - dyskinetic
- caused by damage to the basal ganglia and the substantia nigra
- athetoid movements and oro-motor problems - ataxic
- caused by damage to the cerebellum with typical cerebellar signs - mixed
Up to what age is bow legged normal?
< 3 years and usually resolves by 4
Barlow vs ortilani?
Barlow test: attempts to dislocate an articulated femoral head
Ortolani test: attempts to relocate a dislocated femoral head
How does HUS present?
- AKI
- Thrombocytopaenia
- normocytic anaemia
- brief gasteronentritis episode (e-coli)
What is a common complication when taking amoxicillin for infectious mono?
maculopapular pruritic rash
What would blood gas/blood tests for DKA show?
- metabolic acidosis
- hyperkalaemia (due to acidosis and lack of insulin meaning lots of potassium remains in the blood rather than being moved into cells)
- Hyperglycaemia
- ketonaemia
- Mildly raised creatinine
- Low bicarbonate (used by ketones)
- Slightly raised sodium (due to dehydration)
How does an adrenal crisis caused by CAH present?
- Hyperkalaemia and hyponaetremia
- metabolic acidosis
- Raised 17-hydroxyprogesterone
Most common organism that causes glandular fever? and how does it present
EBV
Fever
Sore throat/tonsillar exudate
Fatigue
Hepatomegaly and/or splenomegaly may sometimes be found on palpation
heterophile antibody ‘Paul Bunnell’ test
What is considered sanctuary sites for chemo?
CNS (due to BBB) and testes
What medications are used for sickle cell disease?
- Prophylactic penicillin due to splenectomy
- Hydroxycarbamide to prevent vaso-occulsive complications
Difference between haemophillia A vs B?
A: Factor VIII
B: Factor IX
What are the complications of Kawasaki disease?
- Coronary artery aneurysm
- Pericardia effusion
- Myocardial disease/valve damage
Do a transthoracic echo
How does Kawasaki present?
CRASH AND BURN
Conjunctivitis, Rash, Adneopathy,
Strawberry tongue, Hands (palmar erythema, swelling), Burn (fever >5 days)
What are the complications of shingles?
Ramsay hunt syndrome
What are the complications of mumps?
Pancreatitis
Orchitis
Meningitis
Sensorineural hearing loss
What are the notifiable diseases?
- MMR
- HUS
- Whooping cough
How is cancer treatment response monitored?
PET scan -> shows areas of high uptake and therefore malignancy
Which lymphoma is more common in childhood?
non-hodgkins
What are the B symptoms seen in lymphoma?
unexplained fever, unexplained weight loss, and drenching sweats (particularly at night)
What are the risk factors for DDH?
Female, Breech birth, high birth weight, oligohydramnios, and prematurity
What condition would you find a ‘double bubble’ sign-on x-ray? and what genetic condition is it associated with?
Dudodenal atresia - downs (has billous vomitting)