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1
Q

Cystic fibrosis treatment

A
Chest physio
High calorie diet
CREON tablets for pancreatic enzymes
Prophylactin flucox and vaccinations
Nebulised dornase alfa
Nebulised hypertonic saline
Fertility treatment
Genetic counselling
2
Q

If you have two parents who are CF carriers, whats your chance of getting it

A

25%

3
Q

What causes breathlessness in anaphylaxis

A

Angio oedema

4
Q

Whats the best investigation for cystic fibrosis

A

Sweat test

5
Q

Right illiac fossa tenderness

A

IBD

6
Q

What type of anaemia do you get from GI blood loss

A

Microcytic

7
Q

What happens to albumin in diarrhoea

A

Low because lost in stool and poorly absorbed because of malabsorption

8
Q

Best investigation for inflammatory bowel disease

A

Colonoscopy

9
Q

Features of both UC and crohns

A
Diarrhoea
Abdo pain
Weight loss
N and V
Faltering growth
Delayed puberty
Reduced appetite
Malaise/ fatigue
Fever
10
Q

Features of IBD unique to crohns

A

Termina ileum disease (RLQ pain)
Perianal tags, fistulae, abscesses
Gallstones

11
Q

Features of IBD unique to UC

A

Left lower quadrant tenderness

Bloody diarrhoea

12
Q

Macroscopic and microscopic crohns features

A

Skin lesions, cobblestone mucosa

non caseating granulomata, transmural inflammation

13
Q

Which IBD do you get toxic megacolon in

A

UC

14
Q

Macroscopic and microscopic UC features

A

Continous, mucosal ulceration. No granulomas. Submucosal inflammation

15
Q

Crohns induction of remission treatment

A

Enteral nutrition (modulen) then glucocorticoids

16
Q

Crohns maintenance of remission treatment

A

Azathioprine

17
Q

UC induction of remission treatment

A

Mesalazine or glucocorticoids

18
Q

Treatment of toxic megacolon

A

IV, fluids, glucocorticoids, surgery

19
Q

Maintenance of remission in UC

A

Mesalazine

20
Q

Define coeliac

A

Autoimmune condition caused by an inflammatory response by the GALT to gliadin (HLA-DR3-DQ2)

21
Q

Which antibodies are present in coeliac

A

Tissue transglutaminase

Endomysial cell antibodies

22
Q

Diagnosis of coeliac

A

Dont eat gluten for 6 weeks then test for antibodies then endoscopy and biopsy

23
Q

Microscopic changes in coeliac

A

Long crypts, lymphocyte infiltration, flattened villi

24
Q

Magement of coeliac

A

Gluten free diet

25
Q

Symptoms of coeliac

A
Unintended weight loss
Fatigue
Chronic diarrhoea
Flatulence
Severe recurrent abdo pain
Pale stools
26
Q

Causes of gastroenteritis

A

Virus ! (rotavirus, adenovirus, enterovirus)

Bacterial- campylobacter jejuni, E. coli, shigella, salmonella

27
Q

Clinical features of gastroenteritis

A
Acute onset diarrhoea and vomitting
Fever
Lethargy
Abdominal pain
Poor feeding
28
Q

If watery diarrhoea returns after gastroenteritis whats going on

A

Post gastroenteritis syndrome (may have developed lactose intolerance)

29
Q

When should you take stool samples in gastroenteritis

A
Not sure of diagnosis
Septic
Bloody
More than 2 weeks
Immunocompromised
30
Q

Management of gastroenteritis

A

Oral rehydration solution
Breast feed
NG fluids
Hospitalisation if needed

31
Q

Common symptoms of appendicitis

A

RLQ pain, worse on movement, Vomiting, fever, peritonism

32
Q

Diagnosis of appendicitis

A

Clinical diagnosis

Ultrasound

33
Q

Treatment of appendicitis

A

Appendicectomy

34
Q

Common symptoms of intussusception

A

Sudden colicky pain, drawing up legs, pale, vomiting, redcurrent jelly stools

35
Q

Diagnosis of intussusception

A

Target sign on ultrasound

36
Q

Management of intussusception

A

IV fluids and antibiotics
Pneumotic reduction air enema
Laparotomy

37
Q

Common symptoms of mesenteric adenitis

A

Mimics appendicitis, pharyngitis, cervical lymphadenopathy

38
Q

Treatment of mesenteric adenitis

A

Analgesia

Hydration

39
Q

Common symptoms of inguinal hernia

A

Reducible lump in groin, if strangulated= nausea, vomiting, off food, sever pain

40
Q

Investigation of inguinal hernia

A

Clinical exam- rule out testicular torsion

41
Q

Treatment of inguinal hernia

A

Early surgical intervention

42
Q

Symptoms of irritable bowel syndrome

A

Pain relieved on defecation, bloating, mucus in stool, lethargy

43
Q

Treatment of irritable bowel

A

Small regular meals, eliminate triggering foods

44
Q

Symptoms of abdominal migraine

A

Pain lasting 2-72 hours, nausea, vomiting, anorexia, pallor

45
Q

Treatment for abdominal migraine

A

Analgesia, avoid triggers, prophylaxis

46
Q

What type of bilirubinaemia causes jaundice in the first 24 hours

A

Unconjugated

47
Q

Causes of jaundice in first 24 hours

A
Haemolytic disease of the newborn
Hereditary spherocytosis
G-6-PD deficiency
Sepsis
TORCH infections
Crigler-najjar syndrome
48
Q

Causes of jaundice after 2 weeks

A

Biliary atresia
Hypothyroidism
UTI
Gilbert syndrome

49
Q

Jaundice investigations

A
Bilirubin chart
FBC
Blood film
Unconjugated and conjugated 
Blood typing of mother and baby
Direct Coombs test
50
Q

What does the direct coombs test look for

A

Haemolysis

51
Q

How does the molecule go from haem to conjugated bilirubin

A

Haem
Biliverdin
Unconjugated Bilirubin
Conjugated bilirubin

52
Q

What is biliary atresia

A

Narrowing blockage or absence of part of the biliary tree. Leads to a conjugated bilirubinaemia

53
Q

Presentation of biliary atresia

A

severe jaundice at day 2 (high conjugated), pale white stools

54
Q

Biliary atresia treatmtent

A

Ultrasound of gall bladder and bile ducts
TBIDA radioisotope scan
Kasal surgery

55
Q

Anti D injections

A

Given at 28 weeks and again at birth or after a sensitisation event

56
Q

Kliehauer test

A

Assess how much fetal blood mixed with the mothers blood

57
Q

Hereditary spherocytosis presentation

A

Newborn with jaundice and splenomegaly

58
Q

Describe hereditary spherocytosis pathology

A

Autosomal dominant. Sphere shaped red blood cell.

Aplastic crisis if also parvovirus

59
Q

Investigations of spherocytosis

A

FBC, Blood film, Coombs test

60
Q

Treatment of spherocytosis

A

Splenectomy
Cholecystectomy
Folate
Penicillin for life

61
Q

G6PD deficiency pathology

A

Baby picks up infection and turns yellow. X linked recessive.

62
Q

G6PD triggers and presentation

A

Infection, medication or broad beans causes splenomegaly and gall stones

63
Q

Investigations of G6PD deficiency

A

Heinz bodies on blood film

G6PD enzyme assay

64
Q

G6PD fun fact

A

Caused by fava beans and Heinz bodies is a sign of it

65
Q

Gilberts syndrome

A

Unconjugated bilirubin. Cos cant conjugate bilirubin. Jaundice only during illness, exercise or fasting

66
Q

Crigler Najjar

A

Jaundice at birth, death by 2.

67
Q

Neonatal hepatitis presentation

A

IUGR, jaundice, failure to thrive, itchy rash, dark urine and hepatomegaly

68
Q

Neonatal hepatitis investigation

A

Liver biopsy shows multinucleated giant cells and rosette formation
Derranged LFTs

69
Q

What is breast feeding/ physiological jaundice

A

Appears day 3, peaks at 7 then goes.
Foetal Hb has shorter lifespan and newborn liver cant cope.
Can randomly happen more in breastfed

70
Q

Jaundice, hypotonia and large tongue

A

Hypothyroidism (ioidine, hormonal dysgenesis, autoimmune thyroiditis later in childhood)

71
Q

Key symptoms of hypothyroidism

A

Delayed puberty and development, puffy face, macroglossia, short stature, cretinism

72
Q

Hypothyroidism investigations

A

Heel prick test, ultrasound of the neck, TSH levels high

73
Q

Treatment of hypothyroidism

A

Levothyroxine

74
Q

Galactosaemia presentation

A

Jaundice, vomiting and poor feeding worsened by cows milk feeding. GALT enzyme missing.
Cataracts

75
Q

Galactosaemia treatment

A

Both mum and baby need to stop dairy. Breastfeed only

76
Q

Kernicterus

A

Convulsions, lethargy, poor feeding, coma, cerebral palsy, learning difficulties and death because unconjugated bilirubin has crossed the BBB

77
Q

Wilsons disease pathology

A

Reduced caeruloplasmin.

Kayser fleischer rings, parkinsonism, rickets, jaundice

78
Q

Investiagtions of wilsons disease

A

Increased 24hr copper urine, reduced serum caeruloplasmin. Penicillamine and zinc treatment

79
Q

Alpha 1 antitrypsin

A

Less A1AT so more neutrophil elastase. Neonatal jaundice and bleeding. Worse with breastfeeding. Look for serum levels. Make sure they dont smoke

80
Q

Febrile seizures

A

Seizure in association with fever with no definable intracranial cause.

81
Q

What is status epilepticus

A

Over 30 mins

82
Q

What classes as a simple febrile seizure

A

Generalised tonic clonic lasting up to 24 hours and not recurring within 24 hours

83
Q

Treatment of febrile seizures

A

Paracetamol and ibuprofen, education and reassurance

84
Q

What do you call seizures that happen in sleep

A

Benign neonatal sleep myoclonus or parasomias

85
Q

What do you call seizures that happen when feeding

A

Sandifer syndrome/ GORD

86
Q

Describe syncope

A

Older child, prodromal pallor, rapid recovery. Situational

87
Q

Neonatal life support

A

Dry the baby
Get help, open airway, feel for breathing, 5 rescue breaths (cover nose holes, neutral neck position). Check for signs of circulation.
15 chest compressions (tips of fingers):2 rescue breaths

88
Q

Where do you check for circulation in a newborn

A

Brachial and femoral

89
Q

APGAR what does it stand for

A
Activity (muscle tone)
Pulse
Grimace (reflex irritability)
Appearance (skin colour)
Respiration
90
Q

What are the scores for activity in apgar

A

absent=0, 1= flexed limbs, 2= active

91
Q

What are the scores for pulse in apgar

A

absent=0, 1= less than 100, 2=more than 100

92
Q

What are the scores for grimace in apgar

A

floppy=0, 1=minimal response, 2=prompt response

93
Q

What are the scores for appearance in apgar

A

Blue, pale=0, pink body, blue extremities=1, pink=2

94
Q

What are the scores for respiration in apgar

A

absent=0, slow and irregular=1, virgorous cry=2

95
Q

Whats a very low apgar

A

Below 3

96
Q

Whats a good apgar

A

Above 7

97
Q

When should you do apgar

A

1, 5 and 10 minutes

98
Q

What is on the guthrie card/ heel prick test

A
Congential hypothyroidism
Cystic fibrosis
Sickle cell disease
Phenylketonuria
MCADD
MSUD
IVA
GA1
HCU
99
Q

What are the neonatal infections

A
TORCH
Toxoplasmosis
Rubella
CMV
Herpes simplex
100
Q

Toxoplasmosis neonatal

A

Hydrocephalus, cerebral palsy

101
Q

Rubella neonatal

A

Sensorinueral deafness, growth retardation, cerebral palsy, purprual skin lesions

102
Q

Herpes simplex neonatal

A

Limb hypoplasia and corticol atrophy- give baby Varicella Zoster Immune globulin (VZIG)

103
Q

Transient tachypnoea of newborn

A

After emergency C section. Physiological pulmonary oedema. CXR= hyperinflation of the lungs and fluid in the horizontal fissure.
Should resolve by itself

104
Q

Respiratory distress syndrome

A

Early. Tachypnoea, intercostal recession and is cyanosed. CXR= ground glass appearance, indistinct heart border and air bronchograms.
Give mum dexamethasone and baby surfactant and oxygen

105
Q

Meconium aspiration syndrome

A

post term, mum smokes and has HTN. Meconium stained amniotic fluid.
CXR= Patchy infiltrates, atelectasis, coarse streaking of both lungs.
Suctioning is the treatment

106
Q

Neonatal sepsis (group B strep)

A

Premature and prolonged ROM.
Fever, reduced tone and activity. RDS. Hypoglycaemia.
BenPen and Gentamicin.
Then blood cultures, CRP and LP

107
Q

Persistent pulmonary hypertension of newborn

A

Failure of transition to adult circulation. Respiratory syndromes, Maternal SSRI or aspirin.

108
Q

Chronic lung disease of prematurity/ bronchopulmonary dysplasia

A

Still requires oxygen 28 days after delivery.
Scarring and reduced alveolar surface area.
Resp symptoms.
CPAP and caffeine

109
Q

Apnoea of prematurity

A

Premature baby stops breathing for more than 20s.
IV caffeine
Tactile stimulation when alerted by apnoea monitors

110
Q

Intraventricular haemorrhage

A

Premature babies within first 3 days. Go into shock, hydrocephalus. Vitamin K is the treatment

111
Q

Cephalohaematoma

A

Several hours after birth. Doesnt cross suture lines, blood under parietal region.

112
Q

Caput succedaneum

A

Present at birth, forms over vertex and crosses suture lines. Resolves in days

113
Q

Neonatal seizure causes

A
Hypoxic ischaemic encephalopathy
Intracranial haemorrhage
Meningitis
Tuberous sclerosis
Fetal withdrawal syndrome
114
Q

Hypoxic ischaemic encephalopathy

A

Asphyxia at birth
Sarnat staging system
Therapeutic hypothermia treatment

115
Q

Necrotising enterocolitis presentation and pathology

A

Bacterial invasion of ischaemic bowel. Feeding intolerance, distended abdomen, bloody stools and green vomit

116
Q

Investigation and treatment of NEC

A

Dilated bowel loops,
air in portal tract,
air under diaphragm, intramural air. Riglers and football sign.
Nil by mouth, IV fluids, IV broad spectrum abx and laparotomy

117
Q

Retinopathy of prematurity

A

Retinal blood vessel formation is stimulation by hypoxia. So is prevented by too much oxygen.
Scar, detach, blind.
Treat with transpupillary diode laser therapy

118
Q

Congenital diaphragmatic hernia

A

RDS, heart sounds louder on the right, tinkling bowel sounds.
Bochdalek hernia.
NG feed, intubate and ventilate then surgery

119
Q

Whats the difference between gastroschisis and omphalocele

A

Gastroschisis has no peritoneal layer whereas the omphalocele protrudes into umbilical cord

120
Q

Initially baby seems fine but then collapses and turns blue.

A

Transposition of the great arteries, happens when ductus arteriosis closes.
Echo
Prostaglandin E2
Arterial switch procedure

121
Q

Coarctation of the aorta

A

Newborn is floppy, grey and tachypnoeic. Weak femoral pulses and radiofemoral delay.
Do 4 limb blood pressure
Prostaglandin E2 then surgery

122
Q

How does cyclooxygenase inhibition affect the phyioslogical changes within the first 48 hours of birth

A

Promotes ductus arteriosus closure

123
Q

Neonatal cyanosis causes

A
Tricuspid atresia
Transposition of the great arteries
Tetralogy of fallot
AVSD
Eisenmenger syndrome
124
Q

Non blanching rash causes

A
Meningitis
ALL
Congenital bleeding disorders
ITP
HSP
Non accidnetal injury
125
Q

Henoch Schonlein Purpura

A

Purpuric non blanching rash on buttocks and legs, abdo pain and joint pain.
IgA vasculitis
Skin, kidneys, GI
Self limiting

126
Q

Kawasaki disease

A

Fever over 5 days and conjucitivitis, tongue and mucous membranes, desquamification, cervical lymphadenopathy
Aspirin and IV IG
Coronary aneurysms

127
Q

Meningococcal sepitcaemia

A

Blood culture and LP, send blood for meningococal PCR
IM BenPen
Cefotaxim
Less than 3 months= add amoxicillin for Strep
Give dexamethasone too

128
Q

Rheumatic fever

A

After tonsilitis.
Swollen large limbs, heart infection, murmurs and pericardial rub.
Strep pyogenes.
Throat swab.
NSAIDs and long term prophylactic Abx to prevent endocarditis

129
Q

Whats the criteria called for rheumatic fever

A

JONES

Joint, heart, nodules, erythema marginatum, sydenham chorea

130
Q

Chicken pox (varicella zoster virus)

A

Vesicular rash. Blistering. Starts and trunk or face.
Keep cool, trim nails.
Varicella Zoster Immunoglobulin (VZIG) if newborn

131
Q

Shingles (herpes zoster virus)

A

Acute, unilateral painful blistering rash in dermatomal distribution.
Can cause pneumonia and encephalitis.
Aciclovir
(VZIG for non immune mothers)

132
Q

Candida

A

Inflamed rash, satellite lesions and oral lesions. Topical imidazole. Cease the use of a barrier cream.

133
Q

Nappy rash

A

Dont use talcum powder.

Leave nappy off as much as possible, dry bottom, change regularly. Use a mild hydrocortisone

134
Q

Impetigo

A

Exfoliative toxins made by staph aureus. Golden crust. Swabs for diagnosis.
Topical fusidic acid
Oral flucoxacillin

135
Q

Erythema infectiosum (slapped cheek)

A
Non specific symptosm and slapped cheek red rash. 
Parvovirus B19. 
Spreads to upper arms.
Foetal hydrops. 
Sickle cell can turn aplastic crisis.
136
Q

Roseola infantum

A

High fever, maculopapular rash and convulsions. (starts on chest)
Human herpes 6

137
Q

Scarlet fever

A

sandpaper rash on torso, spares the face, strawberry tongue
S pyogenes
Penicillin V
Swab throat

138
Q

Hand foot and mouth disease

A

Coxsackie A16 virus
Blistering red spots on hands feet and mouth after normal cold.
Very contagious but self limting

139
Q

Staphylococcal scalded skin syndrome

A

Nikolskys sign- seperation of skin on gentle pressure.
S. Aureus bacteria produces exfoliative toxins.
Flucoxacillin
Fusidic accid
fluid and electrolyte management
Paracetamol

140
Q

Toxic shock syndrome

A

Staphylococcal exotoxin
Leaving tampons in too long.
High fever, signs of shock, diffusion desquamating erythematous rash.
ABCDE, oxygen, IV broad spectrum abx, IVIG

141
Q

Measles

A

Prodrome, koplik spots (buccal mucosa), rash starts behind ears

Encephlaitis, otittis media, panencephaltiis.

Give MMR. You have immunity from mum until 9 months

142
Q

Erythema multiforme

A

Widespread target lesions. Pyrexia, stomatitis, muscle and joint aches.
Hypersensitivity reaction.
Resolves.

143
Q

Molluscum contagiosum

A

Viral. Flesh coloured papules with a central dimple.

Salicylic acid

144
Q

Scabies

A

Incredibly itchy, between the fingerwebs then whole body.
Sarcoptes scaibiei.
Permethrin cream.
Wash everything

145
Q

Ringworm

A

Itchy circular erythematous and scaly well demarcated rash.
Fluconazole orally
Miconazole cream.
Steroids make fungal infections worse

146
Q

Seborrhoeic dermatitis

A

Cradle cap

147
Q

Eczema

A

Dry, red, itchy over flexor surfaces in infancy then extensor in young children.

Emolients and special soaps, avoid triggers.
Wet wraps
Hydrocortisone for as little time as possible (skin thinning and telangiectasia)

148
Q

Psoriasis

A

Scaly, dry, flakey, raised and rough on extensor surfaces. Keratinocyte proliferation.
Topical corticosteroids and vitamin D analogues

149
Q

Salmon patch

A

Flat red or pink on face. Fade in a couple of months

150
Q

Infantile hemangioma

A

Strawberry marks. Rapidly increase in size for first 6 months then shrink and disappear by 7 years

151
Q

Capillary malformation

A

Port wine marks.
Unilaterally normally on face, chest and back.
Permanent and receptive to hormones.

152
Q

Cafe au lait spots

A

More than 6 before 5 could be neurofibromatosis

153
Q

Mongolian spots

A

Lower back or buttocks. Darker skinned. Dissapear by age 4

154
Q

Congenital meloncytic naevi

A

Normal moles

155
Q

Slipped upper femoral epiphysis

A

Obesity, puberty growth surge.
Limping, pain on movement in all directions.
Management= immobilisation, analgesia, screw fixation

156
Q

4-10 year old limb

A

Transient synovitis
Perthes disease
JIA

157
Q

Septic arthritis

A
Streptococci neonatally
Staph aureus afterwards
Acute pain and swelling, fever, rigors. 
Kocher modified criteria. 
Joint aspiration, blood cultures, splinting, IV abx, surgical drainage
158
Q

Developmental dysplasia of the hip

A

Barlow and ortolani tests
Painless limp, limited abduction, assymetrical skin folds.
Family history, breech, oligohydramnios.
Ultrasound and plain radiograph

159
Q

Perthes disease

A

Avascular necroissi of the femoral head.
Limp in the absence of trauma. groin pain, limited rom, muscle wasting.
Pain relief, physio or surgery

160
Q

Transient synovitis

A

Sudden onset limp. No rest pain. Well child. Blood and X Ray.

Rest and simple analgesia

161
Q

JIA

A

Joint inflammation presenting before 16 for atleast 6 weeks. With absence of cause.
Systemic JIA- temperatures and salmon pink rash.

Physio, NSAIDs, intra articular corticosteroid injections