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MSRA > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

Meningitis management <3 months and >3 months

A

<3 months= IV amoxicillin and cefotamine

> 3 months= IV cefotaxime (ceftriaxone) +/- dexamethasone if LP shows:
- WCC high, bacteria, or purulent CFS

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2
Q

Shaken baby triad (3)

A

Subdural haemorrhage
Retinal haemorrhage
Encephalopathy

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3
Q

Pethers (ischaemia) investigations (1)

A

X-ray
shows widening join spaces

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4
Q

Pethers management (1) when to operate? (1)
what are they at risk of? (1)

A

<6 years= conservative
>6 years= surgical consideration

Risk of OA

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5
Q

Scarlet fever is spread via respiratory route by
- what bacteria? (1)
- what management? (1)

A
  • Group A haemolytic streptococci (usually Streptococcus pyogenes)
  • 10/7 penicillin
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5
Q

Scarlet fever:
- when return to school? (1)

A

within 24hr
IT IS NOTIFIABLE DISEASE

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6
Q

Scarlet fever:
complications? (4)

A
  • otitis media: the most common complication
  • rheumatic fever: typically occurs 20 days after infection
  • acute glomerulonephritis: typically occurs 10 days after infection
  • invasive complications (e.g. bacteraemia, meningitis, necrotizing fasciitis) rarer
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7
Q

Paediatric migrane:
management? (2)

A
  • 1st line= ibuprofen
  • 2nd line IF OVER 12 = triptans
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8
Q

Downs syndrome:
- most common cardiac abnormality? (1)
- haematological? (1)

A
  • Atrioventricular septal defect
  • ALL
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9
Q

Purulent discharge and conjunctival inflammation in <30 days old makes you think of… (1)
- management (1)
- responsible organisms (2)

A

ophthalmia neonatorum (conjunctivitis under 30 days)

–> REFER OPTHALOMOGY

Responsible organisms include
- Chlamydia trachomatis
- Neisseria gonorrhoeae

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10
Q

Hirschprung’s disease:
- management 1st line? (1)
- 2nd line? (1)
- associated with with congenital abnormality (1)

(aganglionic segment of bowel due to a developmental failure of the parasympathetic)

A
  • 1st= rectal washouts/ bowel irrigation
  • 2nd= surgery
  • Downs syndrome
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11
Q

Roseola infantum:
- age (1)
- main features? (2)
- school exclusion? (1)
- organism? (1)

A
  • 6 months - 2 years
  • fever followed later by rash 1-2 weeks later, erythematous across limbs and trunk
  • febrile seizures common
  • no school exclusion
  • human herpes virus 6 (HHV6)
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12
Q

Rubella:
- rash pattern? (1)
- other features (3)

A

starting on the face before spreading to the rest of the body

  • mild fever, sore throat and lymphadenopathy.
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13
Q

Croup:
- moderate features (5)
- severe features (5)

A

MODERATE:
- Frequent barking cough
- Easily audible stridor at rest
- Suprasternal and sternal wall retraction at rest
- No or little distress or agitation
- The child can be placated and is interested in its surroundings

SEVERE:
- Frequent barking cough
- Prominent inspiratory (and occasionally, expiratory) stridor at rest
- Marked sternal wall retractions
- Significant distress and agitation, or lethargy or restlessness (a sign of hypoxaemia)
- Tachycardia occurs with more severe obstructive symptoms and hypoxaemia

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13
Q

Measles:
- rash pattern? (1)
- other features? (4)

A

starts behind the ears and then spreads to the rest of the body.

4Ks:
It is associated with fever, conjunctivitis, coryzal symptoms and white koplik spots on the inside of the mouth.

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14
Q

Croup:
- management (1)
- age (1)
- when to admit? (1)

(stridor, fever, coryza, increased WOB)

A
  • Single dose of oral dexamethasone (0.15 mg/kg) is to be taken immediately regardless of severity
  • 6 months - 1 year
  • admit if ANY moderate/ severe Sx
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15
Q

Croup:
investigations/ diagnosis? (2)

A
  • clinical diagnosis
  • XR chest sometimes –> STEEPLE sign anterior view, THUMB sign in lateral view
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16
Q

Bow legs:
<4 yrs management? (1)

A
  • Bow legs in a child < 3 is a normal variant and usually resolves by the age of 4 years
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17
Q

Risk factors for surfactant lung disease (4)

A

male sex
diabetic mothers
Caesarean section
second born of premature twins

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18
Q

Caffeine in babies

A

can be used as a respiratory stimulant in newborn babies

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19
Q

Surfactant lung disease:
- CXR finding (1)
- management (4)

A
  • ‘ground-glass’ appearance with an indistinct heart border
  • corticosteroids to mum
  • O2
  • ventilation
  • exogenous surfactant via ET tube
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20
Q

nocturnal enuresis:
- what age?

A

BEFORE 5 (so 3-4 years)

rewards –> enuresis alarm –> desmopressin

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21
Q

Differential of: non-blanching petechial rash but NO fever (2)

A

ITP (recent cold)

AKA Immune (or idiopathic) thrombocytopenic purpura (ITP) is an immune-mediated reduction

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22
Q

HUS triad (3)

A

microangiopathic haemolytic uraemia
acute kidney injury
thrombocytopenia

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23
Q

Meningitis organisms
- <3 months
- 1month - 6 years
- >6 years

A

Neonatal to 3 months
Group B Streptococcus: usually acquired from the mother at birth. More common in low birth weight babies and following prolonged rupture of the membranes
E. coli and other Gram -ve organisms
Listeria monocytogenes

1 month to 6 years
Neisseria meningitidis (meningococcus)
Streptococcus pneumoniae (pneumococcus)
Haemophilus influenzae

Greater than 6 years
Neisseria meningitidis (meningococcus)
Streptococcus pneumoniae (pneumococcus)

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24
Q

Vitamin K in babies: who is deficient?

A

breastfed

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25
Q

Raised FSH/LH in primary amenorrhoea

A

Turners syndrome (gonadal dysgenesis)

WOULD PRESENT EARLY

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26
Q

Raised FSH, LH and low oestradiol with secondary amenorrhaea

A

premature ovarian failure

(cessation of menses for 1 year before the age of 40)

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27
Q

intrauterine adhesions following dilation and curettage
+ secondary amenorrhoea

A

Asherman’s syndrome

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28
Q
A
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29
Q

Primary amenorrhoea, little or no axillary and pubic hair, elevated testosterone

A

androgen insensitivity syndrome

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30
Q

development of male secondary sexual characteristics in females (such as deep voice and hirsutism)

A

Congenital adrenal hyperplasia (CAH)

impaired cortisol synthesis leading to surplus progesterone which is converted to extra testosterone to reduce the levels of progesterone. Unlike AIS, the body is still responsive to testosterone, therefore, this would mean that this patient would have hirsutism and excess male-pattern hair growth, including axillary and pubic hair, which is not seen here. A diagnosis of CAH would also not explain the bilateral lower pelvic swellings, which are likely to be undescended testes.

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31
Q

medication to reduce size of uterine fibroids

A

GnRH agonist e.g. leuprolide

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32
Q

COCP and surgery

A

don’t have 4-6 weeks prior to major surgery

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33
Q
A
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34
Q

best HRT for VTE risk

A

transdermal

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35
Q

Strawberry cervix
- cause
- management

A

Trichomonas vaginalis

Oral metronidazole

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36
Q

bacterial vaginosis

A

oral metronidazole

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37
Q

gonorrhoea management

A

IM ceftriaxonec

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38
Q

cottage cheese discharge
cause?
management?

A

thrust/ vaginal candidasis/ Candida albicans

medication:
oral fluconazole 150 mg as a single dose first-line
clotrimazole 500 mg intravaginal pessary as a single dose if oral therapy is contraindicated
If there are vulval symptoms, consider adding a topical imidazole in addition to an oral or intravaginal antifungal
if pregnant then only local treatments (e.g. cream or pessaries) may be used - oral treatments are contraindicated

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39
Q

vulval erythema, fissuring, satellite lesions may be seen

A

thrush / candida

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40
Q

recurrent thrush treatment?

A

4+ episodes / year = recurrent

confirm with swab
induction: oral fluconazole every 3 days for 3 doses

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41
Q

Most common benign ovarian tumour in women under the age of 25 years

A

Dermoid cyst (teratoma)

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42
Q

The most common cause of ovarian enlargement in women of a reproductive age

A

Follicular cyst

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43
Q

Most common type of ovarian pathology associated with Meigs’ syndrome

A

fibroma

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44
Q

commonest type of ovarian cyst

A

Follicular cysts

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45
Q

If ruptures may cause pseudomyxoma peritonei

A

Mucinous cystadenoma

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46
Q

The most common type of epithelial cell tumour

A

Serous cystadenoma

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47
Q

May contain skin appendages, hair and teeth

A

Dermoid cyst (teratoma)

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48
Q

most common ovarian cancer

A

serous carcinoma

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49
Q

chocolate cyst

A

endometriotic cyst

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50
Q

scarlet fever rash (Streptococcus pyogenes): what type of rash? (1)
where? (1)

A

rough rash
diffuse rash involving cheeks, neck and torso
spares hands
sore throat 2 days before

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51
Q

scarlet fever treatment (Streptococcus pyogenes)

A

oral penicillin V for 10/7

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52
Q

An 18-month-old boy presents to the GP with his mother, who is concerned about a new rash. His mother reports that the rash came on suddenly 1 day ago. His mother recalls he had a cold with a high fever two weeks ago, but this appears to have settled now. Otherwise he is normally fit and well.

On examination, there is an erythematous rash across the child’s trunk and limbs. The rash does not appear itchy and blanches with pressure.

A

Roseola infantum

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53
Q

Small testes in precocious puberty indicate what cause?

A

ADRENAL cause

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54
Q

whooping cough vaccine (pertussus) in pregnancy

A

Women who are between 16-32 weeks pregnant are offered the pertussis vaccine

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55
Q

12yo + joint pain + salmon pink rash

A

Systemic onset juvenile idiopathic arthritis (AKA Still’s disease) has a characteristic salmon-pink rash

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56
Q

features of juvenile idiopathic arthritis

A

Features of systemic onset JIA include
pyrexia
salmon-pink rash
lymphadenopathy
arthritis
uveitis
anorexia and weight loss

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57
Q

how to keep ductus arterioles open

A

Prostaglandin E1

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58
Q

Threadworms symptoms (2)

A
  • perianal itching, particularly at night
  • girls may have vulval symptoms
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59
Q

Threadworms treatment (2)

A
  • Mebendazole single dose
  • TREAT WHOLE HOUSEHOLD

Diagnosis may be made by the applying Sellotape to the perianal area and sending it to the laboratory for microscopy to see the eggs. However, most patients are treated empirically and this approach is supported in the CKS guidelines.

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60
Q

Kawasaki disease

A

5+ DAYS OF FEVER

AND

4/5 of:
- Bilateral conjunctivitis
- Cervical lymphadenopathy
- Polymorphic rash
- Cracked lips/strawberry tongue
- Oedema/desquamation of the hands/feet

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61
Q

Kawasaki management

A

high dose aspirin
(and intravenous immunoglobulin)

Kawasaki disease is one of the few indications for the use of aspirin in children. Due to the risk of Reye’s syndrome aspirin is normally contraindicated in children

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62
Q

kawasaki investigations

A

Echo

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63
Q

kawasaki complications

A

coronary artery aneurysm

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64
Q

Turner’s syndrome is associated with a what murmur

A

ejection systolic (bicuspid aortic valve)

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65
Q

transient tachypnoea of the newborn CXR finding

A

hyperinflation and fluid in the horizontal fissure

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66
Q

Cephalohaematoma vs caput succadaneum

A

CEPHALOHAEMATOMA= Several hours after birth, doesn’t cross suture lines, can take months to resolve, parietal

CAPUT SUCCEDANEUM= presenta t brith, crosses lines, resolves within days

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67
Q

commonest cause of stridor in neonate

A

Laryngomalacia
Congenital abnormality of the larynx.
Infants typical present at 4 weeks of age

(croup is 6 months - 3 years)

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68
Q

Whooping cough treatment

A

azithromycin or clarithromycin if the onset of cough is within the previous 21 day

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69
Q

Labial adhesions treatment? (1)
when to give? (1)

A

oestrogen cream
oney give if recurrent UTIs

It is usually seen in girls between the ages of 3 months and 3 years and can generally be treated conservatively. Spontaneous resolution tends to occur around puberty. It should be noted that the condition is different from an imperforate hymen.

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70
Q

mumps school excusion

A

5 days from onset of swollen glands

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71
Q

An infant with inconsolable crying, drawing legs up to the abdomen associated with pallor, vomiting →

A

intussusception

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72
Q

oftening of the cartilage of the patella
Common in teenage girls
Characteristically anterior knee pain on walking up and down stairs and rising from prolonged sitting
Usually responds to physiotherapy

A

Chondromalacia patellae

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73
Q

Seen in sporty teenagers
Pain, tenderness and swelling over the tibial tubercle

A

Osgood-Schlatter disease
(tibial apophysitis)

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74
Q

Pain after exercise
Intermittent swelling and locking

A

Osteochondritis dissecans

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75
Q

Medial knee pain due to lateral subluxation of the patella
Knee may give way

A

Patellar subluxation

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76
Q

More common in athletic teenage boys
Chronic anterior knee pain that worsens after running
Tender below the patella on examination

A

Patellar tendonitis

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77
Q

neonatal blood spot screening (5-7 days of life)

A

congenital hypothyroidism
cystic fibrosis
sickle cell disease
phenylketonuria
medium chain acyl-CoA dehydrogenase deficiency (MCADD)
maple syrup urine disease (MSUD)
isovaleric acidaemia (IVA)
glutaric aciduria type 1 (GA1)
homocystinuria (pyridoxine unresponsive) (HCU)

78
Q

Barlows test

A

Attempts to dislocate an articulated femoral head.

79
Q

Developmental dysplasia of the hip diagnosis

A

US
however, if the infant is > 4.5 months then x-ray is the first line investigation

80
Q

Developmental dysplasia of the hip management

A

most unstable hips will spontaneously stabilise by 3-6 weeks of age
Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5 months
older children may require surgery

81
Q

cradle cap (seborrhoeic dermatitis) treatment

A

baby shampoo and baby oil

82
Q

most common cause hypothyroidism in kids

A

AUTOIMMUNE

other causes: post total-body irradiation (e.g. in a child previous treated for acute lymphoblastic leukaemia)
iodine deficiency (the most common cause in the developing world)

83
Q

acyanotic heart disease types in kids

A

ventricular septal defects (VSD) - most common, accounts for 30%
atrial septal defect (ASD)
patent ductus arteriosus (PDA)
coarctation of the aorta
aortic valve stenosis

ALL OTHERS ARE CYANOTIC (e.g. tetraology of Fallot, TGA, tricuspid atresia)

84
Q

snoring in kids

A

obesity
nasal problems: polyps, deviated septum, hypertrophic nasal turbinates
recurrent tonsillitis
Down’s syndrome
hypothyroidism

85
Q

roseola infantum most common complication

A

febrile convulsion

86
Q

congenital abnrmalities: Noonan

A

Noonan = no neck./ webbed neck
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis

87
Q

congenital abnrmalities: patau

A

polydactyly (can count to 13 on their hands - chromosome 13)

88
Q

congenital abnrmalities: prada willy

A

prada-willy = hypogonadism

89
Q

congenital abnormalities: Edwards

A

Edward syndrome = Prince Edward has a royal chin - micrognathia, microcephaly, overlapping digits

Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers

90
Q

congenital abnormalities; fragile X

A

xtra large

Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism

91
Q

williams syndrome

A

prince William v friendly
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis

92
Q

continuous murmur

A

patent ductus arteriosus

93
Q

Acrocyanosis

A

a completely normal finding in newborn babies within the first 24-48 hours of life. It is characterised by peripheral cyanosis (bluish discolouration of the hands and feet) with normal central perfusion and normal oxygen saturations. This physiological phenomenon occurs due to peripheral vasoconstriction and the normal transition from fetal to newborn circulation. The presence of good tone, normal oxygen saturations both pre and post-ductally, and absence of other concerning features supports this diagnosis.

94
Q

If a formula-fed baby is suspected of having mild-moderate cow’s milk protein intolerance then…

A

switch to extensive hydrolysed formula

95
Q

Patau syndrome chromosome

A

13

96
Q

chrondromalacia patellae vs osteochonditis dissecans

A

Osteochondritits dissecans = LOCKING

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 - all symptoms described by this patient. It’s most common in adolescents and young adults, particularly those who are active or participate in sports.

Chondromalacia patellae is an incorrect option. While it does present with knee pain that worsens with activity, it typically involves a grinding sensation or clicking sound within the knee joint and is often associated with quadriceps muscle weakness. Additionally, chondromalacia patellae doesn’t usually cause intermittent swelling or locking of the joint.

97
Q

umbilical granuloma: how to treat? (1)

A

an overgrowth of tissue which occurs during the healing process of the umbilicus. It is most common in the first few weeks of life. On examination, a small, red growth of tissue is seen in the centre of the umbilicus. It is usually wet and leaks small amounts of clear or yellow fluid.

  • REGULAR SALT ON WOUND

if this does not help then the granuloma can be cauterised with silver nitrate.

98
Q

Which one of the following is the most common cause of nephrotic syndrome in children?

A

minimal change disease

Minimal change glomerulonephritis nearly always presents as nephrotic syndrome, accounting for 80% of cases in children and 25% in adults. The majority of cases are idiopathic and respond well to steroids

99
Q

nephrotic syndrome features (3)

A

Nephrotic syndrome is classically defined as a triad of
proteinuria (> 1 g/m^2 per 24 hours)
hypoalbuminaemia (< 25 g/l)
oedema

100
Q

At what age would the average child acquire the ability to crawl?

A

9 months

101
Q

cows milk protein intolerance

A

Multi-system involvement
7 months would suggest the new introduction of top up feeds which correlates with the symptoms
Faltering growth along with the multi-system involvement would suggest cows’ milk protein intolerance

102
Q

newborn resuscitation steps

A
  1. DRY BABY
  2. assess HR and RR
  3. 5 inflation breaths
103
Q

hypospadias

A

Hypospadias is a congenital abnormality of the penis which occurs in approximately 3/1,000 male infants. There appears to be a significant genetic element, with further male children having a risk of around 5-15%.

It is usually identified on the newborn baby check. If missed, parents may notice an abnormal urine stream.

Hypospadias is characterised by
a ventral urethral meatus
a hooded prepuce
chordee (ventral curvature of the penis) in more severe forms
the urethral meatus may open more proximally in the more severe variants. However, 75% of the openings are distally located.

104
Q

hypospadias management

A

Hypospadias most commonly occurs as an isolated disorder. However, associated conditions include cryptorchidism (present in 10%) and inguinal hernia.

Management
refer for corrective surgery to be done ~12 months of age
it is essential that the child is not circumcised prior to the surgery as the foreskin may be used in the corrective procedure
in boys with very distal disease, no treatment may be needed.

105
Q

Maternal mortality includes…

A

any death in pregnancy and labour as well as up to six weeks post partum

106
Q

Paediatric BLS

A

give 5 rescue breaths if there are no signs of breathing on initial assessment

107
Q

Autosomal recessive conditions are

A

‘metabolic’ - exceptions: inherited ataxias

Autosomal dominant conditions are ‘structural’ - exceptions: Gilbert’s, hyperlipidaemia type II

108
Q

von willebrand disease: what’s important to note in FH

A

DOMINANT inheritance–> must have family history

109
Q

hemorrhagic disease of newbown is also called..

A

vitamin K deficiency
(–> deficiency of 1972)
–> treatment = IM vitamin K after birth

110
Q

multiple erythematous macular lesions omn forehead, eyes, and upper lip that blanch with pressure

A

Stork mark/ salmon patch

111
Q

another word for port wine stain

A

naevus flammeus

112
Q

what age can:
roll from front-to-back, transfer objects to midline, laugh, turn to voice

A

4 months

113
Q

Erythema toxic neonatorum

A

(ALA toxic erythema of the newborn)
looks like excema of newborn
harmless red rash that appears on newborn skin.
can have pustules/ diffuse blotchy erythema

benign
resolves after 7-14 days

114
Q

8 year old, recurrent nosebleeds, bleeding gums, bruises, low platelets, no organomegaly

A

idiopathic thrombocytopenia purpura (ITP)
autoimmune destruction of platelets

Acute = <6 months
refer to specialist
may treat conservatively or with things like red

115
Q

Atpical UTI in children

A

seriously ill
poor urine flow
abdominal or bladder mass
raised Cr
sepsis
NON-E COLI organism
fail to respond to Abx

–> Increased complication risk e.g. renal scaring or septicaemia

116
Q

HIGH risk features

A

no response to cues
weak, high-pitched crying
grunting
RR >60
decreased skin turgor
bulging dontonel
pale/mottled

117
Q

What age to urgently refer all UTIs

A

<3 months

(>3 months if upper UTI)

118
Q

pyloric stenosis: acidosis or alkalosis? (1)
management (1)

A

hypochloraemic, hypokalaemia metabolic alkalosis

pyloromyotomy

119
Q

early jaundice is always..

A

PATHOLOGICAL
haemolytic disease
infection
haemolytic
maternal autoimmune haemolytic anemia
enzyme deficiecies

120
Q

Risk factors for neonatal jaundice

A

COMMON

low birthweight
breastfed
male
east asian
high altitudes
maternal diabetes

121
Q

when is MMR given

A

1 year
3 year 4 months

122
Q

14 M greyish membrane to phaynx, 38.5oC, trouble swallowing, no vaccines

A

diphtheria

123
Q

“thumbprint sign”

A

epiglottitis

124
Q

“steeple sign”

A

Croup

125
Q

slapped cheek

A

scarlet fever
OR
erythema infectiosum/ “Fifth disease”/ slapped cheek - parvovirus B19

126
Q

palpable symmetrical purpuric rash, GI Sx, join pains, renal involvement

A

HSP
Henoch-Schonlein Purpura

IgA mediated autoimmune hypersensitivity vasculitis of childhood

127
Q

age to kick a ball

A

2 years

128
Q

age to smile

A

0-3 months

129
Q

when to refer if cannot walk

A

18 motnhs

130
Q

when to refer if cannot sit without support

A

12 months

131
Q

when to refer if doesn’t know 2-6 words

A

18 months

132
Q

when to refer if cannot smile

A

10 weeks

133
Q

respiratory distress in neonates features

A

grunting
subcostal/ sternal recession
tracheal tug
flaring of nasal alae

134
Q

bronchiolitis most common cause

A

RSV

135
Q

commonest epilepsy in childhood

A

bening rolandic epilepsy

136
Q

TORCH

A

Toxoplasma gondii
Other agents (Treponema palladium, varicella zoster virus, parvovirus B19, Zika)
Rubella
Cytomegalovirus
Herpes simplex virus

137
Q

IUGR vs macrocosmic babies complications

A

Macrosome= more complications at or after birth (obstructed labour, hypoglycaemia)

IUGR= higher longer term cardiovascular problems (HTN/ coronary heart disease/ T2DM). IUGR babies are stressed due to increased cortisol –> lung maturation and less RDS

138
Q

what failure to thrive is clinically significant

A

> 2 standard deviations
Commenest cause in UK= non organic causes e.g. social problems

139
Q

nappy rash

A

Candida albicans
topical imidazole cream (clotrimazole/ miconazole)
can consider steroids if discomfort

140
Q

abdominal mass in kids –>

A

consider films tumor

141
Q

management RSV (bronchiolitis)

A

oxygen and hydration

142
Q

innocent murmur

A

soft
sitting (goes away on positioning)
silent
splitting of S2
no palpable thrill
systolic

143
Q

33 week premature baby
delivered 4 hr ago
tachypnoeic and grunting

A

respiratory distress syndrome

144
Q

when to use ECMO in kids

A

primary pulmonary hypertension or respiratory distress syndrome (think REPLACING the lungs)

145
Q

duodenal atresia

A

scaphoid abdomen
double bubble sign on US

146
Q

CPR ratio in children

A

15:2

147
Q

calculation to estimate weight in child

A

(age+4) *2

148
Q

complications mumps

A

infertility
pancreatitis

149
Q

whooping cough pregnant women

A

erythromycin (azithromycin/ clarithromycin in other s if onset within 21 days)

150
Q

rockerbottem feet- which congenital abnormality

A

EDWARDS

151
Q

autosomal dominant vs recessive

A

Autosomal recessive conditions are ‘metabolic’ - exceptions: inherited ataxias

Autosomal dominant conditions are ‘structural’ - exceptions: Gilbert’s, hyperlipidaemia type II

152
Q

oligohyhdraminos or polyhydraminos- which is a risk factor for breech

A

OLIGO

153
Q

Abdominal x-rays are useful when diagnosing necrotising enterocolitis

A

dilated bowel loops (often asymmetrical in distribution)
bowel wall oedema
pneumatosis intestinalis (intramural gas)
portal venous gas
pneumoperitoneum resulting from perforation
air both inside and outside of the bowel wall (Rigler sign)
air outlining the falciform ligament (football sign)

154
Q

cause of prolonged jaundice

A

biliary atresia
hypothyroidism
galactosaemia
urinary tract infection
breast milk jaundice
jaundice is more common in breastfed babies
mechanism is not fully understood but thought to be due to high concentrations of beta-glucuronidase → increase in intestinal absorption of unconjugated bilirubin
prematurity
due to immature liver function
increased risk of kernicterus
congenital infections e.g. CMV, toxoplasmosis

155
Q

when is meningitis B vaccine given

A

2, 3 and 12-13 months

156
Q

when to give macrocodes for child pneumonia

A

if chlamydia or mycoplasma suspected

(if influenza suspected then give co-amoxiclav)

157
Q

benign Rolandic epilepsy

A

partial seizures at night

158
Q

anticipation - what does it mean

A

EARLIER age of onset in successive generations

Fragile X (CGG)
Huntington’s (CAG)
myotonic dystrophy (CTG)
Friedreich’s ataxia* (GAA)
spinocerebellar ataxia
spinobulbar muscular atrophy
dentatorubral pallidoluysian atrophy

159
Q

UMBILICAL vs inguinal hernia in kids

A

UMBILICAL = resolve by 2-3 years
inguinal= treat

160
Q

umbilical hernias associated with

A

Associations
Afro-Caribbean infants
Down’s syndrome
mucopolysaccharide storage diseases

161
Q

supravalvular aortic stenosis. The syndromic features include the small upturned nose, long philtrum (upper lip length), wide mouth, full lips, small chin, and puffiness around the eyes

A

williams syndrome

162
Q

all breech babies require…

A

US at 6 weeks for DDH

163
Q

complications chicken pox

A

pneumonia
encephalitis (cerebellar involvement may be seen)
disseminated haemorrhagic chickenpox
arthritis, nephritis and pancreatitis may very rarely be seen

164
Q

Gastroschisis

A

Whereas gastroschisis tends to be a stand alone bowel condition, exomphalos is associated with cardiac and kidney diseases

Management
vaginal delivery may be attempted
newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours

165
Q

Exomphalos (omphalocoele)

A

In exomphalos (also known as an omphalocoele) the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum.

Associations
Beckwith-Wiedemann syndrome
Down’s syndrome
cardiac and kidney malformations

166
Q

high pitched cry, temperature greater than 38C in an infant under 3-month-old

A

red flags –> admit

167
Q

children BLS: ratio and rate of chest compressions

A

15:2

chest compressions should be 100-120/min

168
Q

contraindication to pneumococcal vaccine

A

curent febrile illness

169
Q

Vesicoureteric reflux (VUR) investigation of choice

A

Micturating cystography

170
Q

hand, foot and mouth disease is caused by..

A

Coxsackievirus or Enterovirus.

171
Q

most common congenital heart abnormalities; cyanotic and acyanotic

A
  • cyanotic: TGA most common at birth, Fallot’s most common overall
  • acyanotic: VSD most common cause
172
Q

(peripheral cyanosis around the mouth and extremities) is common in neonates

A

Acrocyanosis
may last 24-48hr

173
Q

bronchiolitis vs CAP in children

A

if you think BRONCHIOLITIs then NO ABC

174
Q

Bartter’s syndrome

A

inherited cause (usually autosomal recessive) of severe hypokalaemia due to defective chloride absorption at the Na+ K+ 2Cl- cotransporter in the ascending loop of Henle. It should be noted that it is associated with normotension (unlike other endocrine causes of hypokalaemia such as Conn’s, Cushing’s and Liddle’s syndrome which are associated with hypertension)

175
Q

age for early secondary sexual characteristics

A

before 8 years in girls and 9 years in boys

176
Q

downs syndrome: hypo or hypertonia

A

hypotonia

177
Q

when to refer undescended testes

A

referral should be considered from around 3 months of age, with the baby ideally seeing a urological surgeon before 6 months of age
orchidopexy: Surgical practices vary although the majority of procedures are performed at around 1 year of age

if bilateral–> URGENT referral

178
Q

lymphocytosis and neutropenia following treatment for suspected recurrent tonsillitis

A

EBV

179
Q

Cryoprecipitate contains

A

factor VIII, fibrinogen, von Willebrand factor and factor XIII

180
Q

which cancer causes massive splenomegaly

A

CML
(crazy massive splenomegaly)

other causes:
myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher’s syndrome

181
Q

hypospenism blood film (1)
what condition is hyposplenism common in? (1)

A

COELIAC DISEASE

target cells
Howell-Jolly bodies
Pappenheimer bodies
sideritic granules
acanthocytes

182
Q

haemoarthrosis in young boy is most likely…

A

Haemophilia

(note vWF also associated with Factor VIII and does not really cause haemoarthrosis)

183
Q

increased risk of VTE in these underlying conditions…

A

malignancy
thrombophilia: e.g. Activated protein C resistance, protein C and S deficiency
heart failure
antiphospholipid syndrome
Behcet’s
polycythaemia
nephrotic syndrome
sickle cell disease
paroxysmal nocturnal haemoglobinuria
hyperviscosity syndrome
homocystinuria

184
Q

e.g. elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom)
massive splenomegaly
hypermetabolic symptoms: weight loss, night sweats etc

A

myelofibrosis

Laboratory findings
anaemia
high WBC and platelet count early in the disease
‘tear-drop’ poikilocytes on blood film
unobtainable bone marrow biopsy - ‘dry tap’ therefore trephine biopsy needed
high urate and LDH (reflect increased cell turnover)

185
Q

isolated thrombocytopenia in a well patient

A

ITP

186
Q

drugs that cause ITP

A

Valproic acid
Methotrexate
Carboplatin
Interferon
Isotretinoin
PanobinostatSmall testes in precocious puberty
H2 blockers and proton-pump inhibitors

187
Q

Small testes in precocious puberty

A

adrenal cause

188
Q

stridor is on

A

inspiration

189
Q

Pyloric stenosis classically acid / alkali

A

hypochloraemic, hypokalaemic alkalosis

190
Q

crawling age

A

9 months

191
Q

meningitis - which Cef Abx?

A

cefTRIazone

192
Q

pregnnacy - when can they have BCG vaccine

A

16-32 weeks

193
Q

Pethers management

A

<6 years= observe
>6 years= surgicalh

194
Q

head injury in shaken baby syndrome

A

SUBDURAL

195
Q

neonatal blood spot test

A

congenital hypothyroidism
cystic fibrosis
sickle cell disease
phenylketonuria
medium chain acyl-CoA dehydrogenase deficiency (MCADD)
maple syrup urine disease (MSUD)
isovaleric acidaemia (IVA)
glutaric aciduria type 1 (GA1)
homocystinuria (pyridoxine unresponsive) (HCU)

MSRA (22 decks)

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