Paeds 3 Flashcards
When is the risk of congenital rubella syndrome highest?
If contracted in first 3 months of pregnancy (earlier = more severe defects).
If contracted >20 weeks gestation –> NO additional risk.
Incubation period of rubella?
14-21 days
What are 3 key cardiac abnormalities that may be seen in congenital rubella syndrome?
1) PDA
2) Pulmonary stenosis
3) VSD
Management of varicella of the newborn?
VZIG +/- aciclovir
What is foetal varicella syndrome caused by?
reactivation of the virus in utero as herpes zoster.
this reactivation ONLY occurs when the foetus is infected with varicella <20 weeks gestation.
What is the most common virus transmitted to the fetus during pregnancy?
Cytomegalovirus
What are 6 key maternal infections that can have foetal complications?
1) Rubella
2) VZV
3) Herpes (HSV)
4) Cytomegalovirus
5) Toxoplasmosis
6) Zika
How can congenital rubella affect hearing?
Sensorineural hearing loss
How can congenital rubella affect eyesight?
Congenital cataracts & retinopathy
What is the biggest risk factor for varicella of the newborn?
Maternal infection occurs <4 weeks from birth (50% risk)
Management of maternal CMV infection in an immunocompetent woman?
No treatment
What are the 3 most commonly noted clinical features in congenital CMV?
1) jaundice
2) petechiae
3) hepatosplenomegaly
When is the risk of congenital toxoplasmosis higher in pregnancy?
The risk is higher LATER in pregnancy
How can foetal CMV be diagnosed prenatally?
Amniocentesis & PCR testing of amniotic sample
Must be carried out AFTER 21 weeks gestation, as functioning foetal kidneys are required for the virus to be excreted into the amniotic fluid.
Infections that can be passed to baby in pregnancy –> TORCH
What are they?
T - toxoplasmosis
O - others (syphillis, hep B, zika)
R - rubella
C - CMV
H - herpes simplex
When is sudden infant death syndrome (SIDS) most common?
At 3 months of age
Surgical mx of paediatric intestinal malrotation with volvulus?
Ladd’s procedure - includes division of Ladd bands and widening of the base of the mesentery.
Location of atopic eczema in infants?
Usually involves the face and flexural surfaces of the body (as opposed to its classical flexural distribution).
What is the only definitive treatment for biliary atresia?
Surgical intervention
Mx of SUFE?
Surgery –> internal fixation across the growth plate
What is used to screen newborns for hearing problems?
Automated otoacoustic emission test.
This involves putting a small soft-tipped earpiece in the outer part of a baby’s ear which sends clicking sounds down the ear. The presence of a soft echo indicates a healthy cochlea.
What further test can be done if the otoacoustic emission test is abnormal?
Auditory brainstem response test
Is oligo or polyhydramnios a risk factor for DDH?
Oligohydramnios –> as it restricts foetal movement and thus normal development of the hip joint.
What is the mx of any child presenting with a limp/hip pain + a fever?
Refer for same day hospital assessment (even if a diagnosis of transient synovitis is suspected) –> to rule out septic arthritis.
Precocious puberty in males is uncommon and usually has an organic cause.
What can be used to help determine the location of the pathology?
Testes size
In precocious puberty in boys, where is the location of the pathology with:
a) bilateral enlargement of testes
b) unilateral enlargement of testes
c) small testes
a) gonadotrophin release from intracranial lesion
b) gonadal tumour
c) adrenal cause (tumour or adrenal hyperplasia)
Why are children with Down’s syndrome prone to snoring & sleep apnoea?
This is due to the low muscle tone in the upper airways and large tongue/adenoids.
There is also an increased risk of obesity which in people with Down’s syndrome which is another predisposing factor to snoring.
Peak age of incidence of bronchiolitis?
3m - 6m
Rate of chest compressions in paeds BLS?
100-120
What organis causes slapped cheek syndrome (erythema infectiosum)?
Parvovirus B19
What age does transient synovitis typically affect?
3-8y
What investigations should be performed in infants <3m with a fever?
1) FBC
2) Blood culture
3) CRP
4) Urine testing (for UTI)
5) CXR (only if resp signs)
6) Stool culture (if diarrhoea)
What is the causative agent of roseola infantum?
HHV-6
All breech babies at or after 36 weeks gestation require what screeing after birth?
US screening for DDH 6 weeks after birth (regardless of mode of delivery)
In paeds BLS, what are the 2 appropriate places to check for a pulse?
1) femoral
2) brachial
What neonatal glucose level indicates the need for 10% dextrose infusion?
<1 mmol/L (whether symptomatic or not)
Mx of ALL cases of neonatal hypoglycaemia that are symptomatic?
IV 10% dextrose infusion
Which condition causes joint pain and swelling and a salmon pink rash?
Juvenile idiopathic arthritis
What orgaism causes threadworms?
Eterobius vermicularis
What are some features of growing pains?
- never present at the start of the day after the child has woken
- no limp
- no limitation of physical activity
- systemically well
- normal physical examination
- motor milestones normal
- symptoms are often intermittent and worse after a day of vigorous activity
At what age does infatile colic usually resolve?
6m
How log after startig Abx can a child with scarlet fever return to school?
24h
What is the 1st line preventative medication to remember for abdominal migraine?
Pizotifen (serotonin agonist)
At what age is encopresis considered pathological?
≥4
At what age is enuresis considered pathological?
≥5
At what age does reflux in babies typically resolve?
1 y/o
What is a test feed done with in pyloric stenosis?
Dextrose water:
- if mass palpable on examination, no further investigations needed
- if not palpable –> US
What are the requirements for diagnosing pyloric stenosis on an abdo US?
(3)
1) Pyloric thickness >4mm
2) Pyloric length >18mm
3) An obstruction preventing the passage of fluid beyond the pylorus, despite gastric peristalsis.
What increases the risk of haemolytic uraemic syndrome?
Abx
Which part of the small intestine is most affected in Coeliac disease?
Jejunum
What is the gold standard for diagnosis of coeliac?
Duodenal biopsy
How long must patients be eating gluten for before testing for coeliac?
At least 6 weeks
What 2 genes are associated with coeliac?
1) HLA-DQ2 (90%)
2) HLA-DQ8
Give some complications of coeliac disease
1) Dermatitis herpetiformis
2) Osteoporosis
3) Infertility
4) Malignancy
5) Anaemia (iron, B12, folate)
6) Failure to thrive
7) Peripheral neuropathy
What feature can indicate intestinal atresia during pregnancy?
Polyhydramnios
How is a definitive diagnosis of pyloric stenosis made?
US –> thickened pylorus
What is the fluid requirement for 24 hours in children?
1st 10kg –> 100ml/kg/day
2nd 10kg –> 50ml/kg/day
Subsequent kg –> 20ml/kg/day
What condition is duodenal atresia associated with?
Down’s syndrome
Are air fluid levels seen on AXR in malrotation w/ volvulus?
Yes
What condition is annular pancreas associated with?
Down’s syndrome
What is a rare but significant complication of rotavirus gastroenteritis?
Toxic megacolon
What will an AXR show in duodenal atresia?
‘Double bubble’ sign –> gas filled distended stomach and duodenum with an absence of distal gas.
What gene is associated with coeliac vs RA?
Coeliac –> HLA-DQ2
RA –> HLA-DR4
What is the surgery called in duodenal atresia?
Duodeno-duodenostomy
Abx in gastroenteritis are only indicated if a specific pathogen (like Shigella or Campylobacter) is isolated.
What is the Abx for Shigella or Campylobacter?
Oral azithromycin
What is the definitive investigation for annular pancreas?
Abdo CT scan
How is a dignosis of intestinal malrotation made?
Upper GI contrast study and USS
Mx of intestinal malrotation?
Treatment is by 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).
What classification system is used for classifying biliary atresia?
Ohi system
Why can biliary atresia cause vitamin K deficiency?
Due to biliary cirrhosis and cholestasis.
The decrease in fat absorption leads to a deficiency of fat-soluble vitamins, such as vitamin K.
Can cause bruising.
When should all children with neonatal jaundice have their bilirubin levels tested?
Within 6 hours
What is the 1st line imaging in biliary atresia?
US
What is the key US finding in biliary atresia?
Triangular cord sign (this is an echogenic sign representing the fibrous remnant of the extrahepatic bile duct) .
Definitive mx of biliary atresia?
Kasai portoenterostomy (removing damaged bile duct and replacing with a loop of intestine).
What is the preferred diagnostic investigation in biliary atresia?
What is the gold standard?
Preferred –> percutanoeus liver biopsy
Gold standard –> operative cholangiography (but only used if uncertainty about diagnosis)
<45 days of life –> provides the best success rate and helps avoid liver transplantation.
<45 days of life
In a severe UC flare, if there has been no improvement after 24h treatment with IV steroids, what should be considered?
Consider adding IV ciclosporin or surgery.
What is the maintenance for UC following a severe relapse or >=2 exacerbations in the past year
Oral azathioprine or mercaptopurine
Are granulomas seen in Crohn’s or UC?
Crohn’s (non-ceseating)
1st line for maintaining remission in Crohn’s?
Oral azathioprine or mercaptopurine
Are continuous lesions seen in Crohn’s or UC?
UC
Mx of perianal fistula in Crohn’s?
If symptomatic –> oral metronidazole
May require surgery
Are crypt abscesses more commonly found in UC or Crohn’s
UC
What will all patients with biliary atresia require for the first year of life post-surgery?
Abx to prevent cholangitis
Which genetic syndrome is most strongly associated with supravalvular aortic stenosis?
William’s syndrome
By what age can a child typically pass objects from hand to hand?
6m
What is the most common complication of roseola infantum?
Febrile convulsions
What syndrome is associated with Hirschsprung’s?
Down’s syndrome
At what age should urgent assessment be arranged in a child presenting with an acute limp?
<3y
School exclusion for scarlet fever?
24h after starting Abx
School exclusion for measles?
4 days from onset of rash
School exclusion for whooping cough?
2 days after commencing antibiotics (or 21 days from onset of symptoms if no antibiotics
What is the most likely result if a fetus is homozygous for alpha-thalassaemia?
Hydrops fetalis –> results in fetal death in utero due to severe anaemia and heart failure.
At what age should there be little or no head lag on being pulled to sit?
3m
How are mitochondrial diseases inherited?
inheritance is only via the maternal line as the sperm contributes no cytoplasm to the zygote
At what age should a child respond to their own name?
5-6m
At what age should a child have a vocabulary of 2-6 words?
12-18m
At what age should a child be able to talk in short sentences (e.g. 3-5 words)?
2.5-3y
What is osteochondritis dissecans?
This condition is characterised by a fragment of bone in the knee joint that becomes detached due to a lack of blood supply.
The fragment and its overlying cartilage can then move around inside the joint, causing symptoms such as pain, swelling, and locking.
When is surgery for hypospadias typically performed?
Around 12m
Mx of Kawasaki disease?
High dose aspirin
How can a germ cell tumour lead to precocious puberty?
Germ cell tumour produces AFP & hCG.
hCG directly stimulates the production of testosterone, oestrogen & sperm.
How is CAH inherited?
Autosomal recessive
Which amino acid increases GH?
Arginine (e.g. red meat, poultry, fish, dairy)
Presentation of severe cases of congenital adrenal hyperplasia?
- Females usually present at birth with virilised genitalia: known as ‘ambiguous genitalia’ and an enlarged clitoris due to the high testosterone levels.
- Hyponatraemia, hyperkalaemia, hypoglycaemia
This leads to:
- poor feeding
- vomiting
- dehydration
- arrhythmias
Which 3 medications normally stimulate the release of GH (and can be used in a GH stimulation test)?
1) Glucagon
2) Arginine
3) Clonidine
Cortisol, ACTH, aldosterone & renin levels in Addisons vs 2ary adrenal failure?
Addison’s –> low cortsiol & aldosterone, raised ACTH & renin
2ary adrenal failure –> low cortisol, low ACTH, normal renin, normal aldosterone
Why is aldosterone normal in secondary adrenal insufficiency?
2ary adrenal insufficiency is due to the absence of the normal stimulation to the adrenal cortex from a lack of ACTH.
This results in a partial or total deficiency of cortisol, but often a normal or near normal production of aldosterone.
Those with 2ary or 3ary adrenal insufficiency will typically have PRESERVED MINERALOCORTICOID function due to the separate feedback system.
What investigation is used to confirm the diagnosis of CAH?
ACTH stimulation
What short synacthen test result indicates 1ary adrenal failure?
Failure of cortisol to rise more than double the baseline.
What is clonidine?
A vasodilator (alpha agonist).
It increases GH.
What medication is used for GH replacement?
Somatropin
Management options for cerebral oedema 2ary to DKA treatment?
1) Slow fluids
2) IV mannitol
3) IV hypertonic saline
Male to male transmission in X-linked recessive conditions?
No male to male transmission
Affected males can only have unaffected sons and carrier daughters.
Is there liver derangement in Gilbert’s syndrome?
No - normal LFTs, isolated raised unconjugated bilirubin
What can be prescribed to breastfeeding mothers whose babies have, or are suspected to have, cows milk protein intolerance?
Calcium & vitamin D (to prevent deficiency whilst they exclude dairy from their diet).
Where does ‘reduced skin turgor’ fall in the NICE traffic light system?
Red flag
What organism causes necrotizing fasciitis?
GAS
What are the 2 types of CFTR modulators that are approved for clinical use in CF?
1) ivacaftor (potentiator)
2) lumacaftor (corrector)
What is the role of CFTR modulators in CF?
CFTR modulators are small molecules that can partially restore function in mutated CFTR.
Describe the prodrome in measles
- mild to moderate fever
- persistent cough
- runny nose
- inflamed eyes (conjunctivitis)
- sore throat
Typically lasts for 2-3 days
What is the recommended compression: ventilation ratio for a newborn?
3:1
3 contraindications of disulfiram in alcohol detox?
1) psyhosis
2) heart disease
2) those felt to be at high risk of suicide
What type of brain haemorrhage may occur spontaneously in premature neonates?
Intraventricular haemorrhage
Most commonly occurs in first 72h after birth.
How can intraventricular haemorrhage result in hydrocephalus?
The blood may clot and occlude CSF flow –> hydrocephalus.
Is DDH more common in males or females?
Females (6x)
give some risk factors for DDH
1) female (6x)
2) breech presentation
3) positive FH
4) firstborn children
5) oligohydramnios
6) birth weight >5kg (macrosomia)
7) congenital calcaneovalgus foot deformity
Which infants require a routine hip US? (3)
1) 1st degree FH of hip problems in early life
2) breech presentation at or after 36 weeks gestation (irrespective of presentation at birth or mode of delivery)
3) multiple pregnancy
Trident hand deformity may be seen in achondroplasia.
What is this?
Short, stubby fingers with separation between the middle and ring fingers.
What is phimosis?
Non-retractile foreskin and/or ballooning during micturition.
If <2 y/o:
- expectant approach
- do NOT forcibly retract foreskin
- personal hygiene
Where is the urethral opening most frequently located in boys with hypospadias?
on the distal ventral surface of the penis
1) Mx of Perthe’s in children <6 y/o?
2) >6 y/o?
1) Reasurrance and follow up
2) Surgical correction
Mx of croup with audible stridor at rest?
Indication for admission
Most common area for intussusception to occur?
Ileocaecal region
Most common ‘lead point’ in intussusception in infants?
Lymphoid hyperplasia (enlargement of lymph tissue) –> these lymph nodes are known as Peyer’s patches (particularly common in ileum).
Viral infection (e.g. rotavirus or norovirus) causes enlargement of Peyer’s patches –> lead point.
What is considered gold standard in diagnosis of intussusception?
Barium enema
What is the leading cause of mortality in those with Hirschsprung’s?
Hirschsprung-associated enterocolitis (HAEC)
How can HSP cause intussusception?
HSP is caused by IgA deposits in small vessel walls.
Intramural hemorrhage and/or inflammation within the mucosa can create lead points that can trigger intussusceptions.
what are some examples of pathological lead points and other secondary causes of intussusception?
1) viral infection –> enlarged Peyer’s patch
2) Meckel’s diverticulum
3) intestinal polyps
4) lymphoma & leukaemia
5) HSP
What is the gold standard investigation for diagnosing malrotation?
Upper GI contrast study
What can cause a mass in the RIF in appendicitis?
Appendix mass –> when the omentum surrounds and sticks to the inflamed appendix, forming a mass in RIF.
What type of volvulus can be associated with neuro conditions e.g. Parkinson’s, Duchenne’s muscular dystrophy?
Sigmoid volvulus
What type of volvulus can be associated with Chagas disease?
Sigmoid volvulus
What type of volvulus can be associated with schizophrenia?
Sigmoid volvulus
What is the most common presentation of malrotation?
BILIOUS vomiting in 1st day of life
1st line Abx in UTI in children?
Oral trimethoprim
When is a DMSA scan done in UTIs?
4-6m after infection
What are some possible underlying causes/triggers in nocturnal enuresis? (4)
1) UTI
2) Diabetes
3) Constipation
4) Stress e.g. bullying
What 3 medications may be indicated in enuresis?
1) Desmopressin
2) Oxybutinin
3) Imipramine (TCA)
What is the most common cause of ambiguous genitalia in newborns?
CAH
