Paediatrics Flashcards

1
Q

What is distal femoral cortical irregularity

A

Distal femoral cortical irregularity has also been referred to as “cortical desmoid”, “cortical irregularity syndrome” and “distal femoral defect”. The typical radiological appearance is a radiolucent cortical defect with adjacent sclerosis. It is often asymptomatic and is almost an incidental finding.

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2
Q

What is choanal atresia?

A

Congenital occlusion of the nasal cavity from the nasopharynx - the ‘choanae’
Maybe due to bone or soft tissue

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3
Q

What is CHARGE syndrome ?

A

Colombo - gap in retina
Heart defects
Atersia of the choanae
Retardation of development
Genitourinary anomalies
Ear anomalies

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4
Q

What are the key features of a pyriform aperture stenosis

A

Piriform aperture < 11mm
Hypoplasia of the hard palate
Medial displacement of the maxillary nasal process - i.e. it moving inwards to make the piriform aperture smaller

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5
Q

What virus causes croup

A

Parainfluenza virus

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6
Q

What virus causes epiglottitis ?

A

H.influenza virus

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7
Q

What is Lemierre syndrome ?

A

Post oral infection - such as tonsillitis. There is extension into the internal jugular vein. This then causes septic pulmonary emboli or mediastinitis - mediastinal extension via the danger space

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8
Q

What bug is responsible for exudative bacterial tracheitis ?

A

S.aureus

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9
Q

What is the most common congenital diaphragmatic hernia ?

A

Bochdalek - back on the left
Causing bowel to herniate
Can result in pulmonary hypoplasia

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10
Q

What is Congenital diaphragmatic hernia associated with ?

A

Bowel malrotation
Neural tube defects
Tuners syndrome
Trisomy 21

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11
Q

What is sequestration?

A

Aberrant lung tissue with systemic blood supply- usually arising from the thoracic aorta
Most common in the left lower lobe

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12
Q

What virus causes bronchiolitis

A

RSV

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13
Q

What typically causes round pneumonia ? what does it look like?

A

S. Pneumoniae - looks like a round pulmonary opacity with well-defined margins, mimicking a mass

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14
Q

What are the causes of bronchiolitis obliterans syndrome ?

A

Final common pathway of small airway obstruction:
Causes include allogenic lung and bone marrow transplant - post lung transplant
Post infectious
Related to toxins / drug exposures

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15
Q

Which infections are associated with swyer-James Macleod syndrome ?

A

Adnovirus and mycoplasma pneumoniae

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16
Q

What is crpytogenic organising pneumonia

A

Filling of the bronchioles and alveoli with granulation tissue polyps -
It is organising pneumonia of unknown cause

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17
Q

What is the atoll sign meant to be associated with ?

A

Cryptogenic organising pneumonia.

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18
Q

What is holt-oram syndrome

A

ASD + upper extremity bone deformities , including abscence or hypoplasia of the thumb

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19
Q

What is the most common ASD malformation ?

A

Ostium secundum - failure of closure of the Ostium secundum with the septum

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20
Q

What are the 4 components of Tetrology of fallot?

A
  1. Overriding Aorta
  2. VSD
  3. RV hypertrophy
  4. Pulmonary atresia
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21
Q

Which syndromes is TOF associated with ?

A

DiGeorge
VACERAL
Trisomy 21

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22
Q

What is the classic CXR finding of TOF and why?

A

Boot shaped - due to RV hypertrophy and reduced pulmonary vascularture.
No cardiomegaly

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23
Q

What is Ebstine anomaly ?

A

Where the right atrium is too low - causing a bigger RA and a smaller RV. This causes reduced pulmonary vasculatture and cyanosis and not enough blood can get too the lungs.

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24
Q

What is the classic CXR finding of Ebstine anomoly ?

A

HUGH box shaped Herat with MASSIVE right atrial enlargement.

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25
Q

What is the most common type of ASD ?

A

Ostium secundum - Caused by incomplete covering of the secundum by the septum.
This one might close without treatment

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26
Q

What are the two most common extracadial shunt lesions resulting in acyanotic congenital heart disease ?

A

Vein of Galen malformation and hepatic hemangioendothelioma.

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27
Q

In paediatric congenital heart disease what is aortic coarctation associated with ?

A

Biscupid aortic valve.
Turners syndrome
Shone syndrome
Intracerebral berry aneurysms

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28
Q

When to patients with Patent ductus arteriosus classically present ?

A

Infants develop CHF around day 7-10 due to vascular resistance falling.

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29
Q

What are the two sub-types of transposition of the great arteries ?

A

Dextrose - TGA
Levo TGA

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30
Q

What is the most common cause of cyanotic heart disease in newborns ?

A

TGA

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31
Q

What are the different subtypes of total anomalous pulmonary venous return ?

A

Supracardiac - pulmonary veins go to left subclavian vein
Cardiac - coronary sinus
Infracardiac - hepatic veins/ portal vein/ hepatic IVC
Mixed

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32
Q

What are the anterior mediastinal masses ?

A

The 4 T’s
- Terrible lymphoma
- Teratoma
- Thymus / Thymoma
- Germ cell tumor

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33
Q

What are the middle mediastinal masses ?

A

Foregut duplication cysts
Neuroenteic cysts
Lymphadenopathy - lymphoma or granulomatous disease.

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34
Q

What are the posterior mediastinum masses?

A

Neurogenic tumours :
neurobloastoma (malignant ) - > ganglioneurobloastoma ->
ganglioneuroma (benign)

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35
Q

What is dose the spinnaker sail sign represent ?

A

Pneumomediastinum

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36
Q

Where should the Umbilical artery catheter be positioned?

A

Terminates ideally at T6-T10 or L3-L5.

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37
Q

What syndrome is rhabdomyoma associated with ?

A

Tuberous sclerosis

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38
Q

Where do cardiac fibroma usually arise ? What is the pathognomic sign?

A

Intraventriclar septum
Central calcification - differentiating them from rhabdomyomas

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39
Q

What is the most common paediatric tumour arising from the cerebral hemisphere ?

A

Astrocymtoma

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40
Q

Where does NEC tend to occur ?

A

Distal ileum and right colon

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41
Q

WHO is at risk of NEC ?

A

Pre-term babies, congenital heart disease, hirschsprung disease and umbilical venous catheter or immunosupression

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42
Q

What is the most common delayed complication of NEC ?

A

Bowel stricture

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43
Q

What are the imaging criteria of pyloric stenosis ?

A

> 3mm thick
Longer than 15mm
No gastric contents passing through the pylorus

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44
Q

What age should you be concerned about a intersusspection ?

A

< 3 months or > 3 years
Need to look for a lead point

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45
Q

What pressure do you inflate to for trying to reduce an intersusseption ?

A

3x 80
3x 100
3 x 120mmHg

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46
Q

How many types of tracheoesophagel fistulas are there ? Which is the most common ?

A

5 types A - E

The most common is oesophageal atresia with distal trachooesophgeal fistula (87%)

Atresia - the oesophagus is just cut in half
B -proximal fistula
C - distal fistula ( most common)
D- Double fistulation ]
E- Just a joining between the two

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47
Q

What are the 3 causes of proximal bowel obstruction - neonate

A
  1. Malrotation mid gut volvulus - surgical emergency
  2. Duodenal artesia
  3. Jejunal atresia
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48
Q

What are the surgical causes of low bowel obstruction ?

A

Hirschsprung disease and distal atresias

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49
Q

What is a micro colon?

A

An abnormally small colon - < 1cm

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50
Q

What is secreted by the small bowel to prevent micro colon?

A

Sulcus entericus

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51
Q

What is one of the earliest manifestations of cystic fibrosis>

A

Meconium ileus - almost 100% of babies with meconium ileus have CF

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52
Q

Group A ependymoma tumours occur in which age group ?

A

Under 5 - group A epdnymoma
Over 5 - group B ependymoma

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53
Q

What is small left colon caused by ?

A

Temporary immaturity of the colonic ganglion cells - causing the distal colon to have abnormal motility right after birth

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54
Q

What are the most common causes of bowel obstruction in children ?

A

AAIIMM
Appendicitis
Adhesions
Inguinal/internal hernia
Meckles diverticulum
Malrotation

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55
Q

What percentage of meckles contains gastric mucosa?

A

25 %

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56
Q

What scan can be done to look for meckles ?

A

Tc-99m Pertechnetate scan

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57
Q

What sign on US might you see in biiliary atresia?

A

Triangular cord sign - echogenic fibrous tissue adjancent tot he portal vein representing abnormal bile duct

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58
Q

What is bilary atresia associated with ?

A

Polysplenia and trisomy 18

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59
Q

How does hepatoblastoma usually present ?

A

Painless abdominal mass with raised AFP

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60
Q

What is usually elevated in hepatblastoma ?

A

AFP > 1000ng/ml

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61
Q

What does a hepatoblastoma look like ?

A

Large, heterogenous, lobulated mass, that takes over the whole liver parenchyma, often cause IVC thrombosis

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62
Q

What age does HCC commonly occur in ?

A

Over 5s

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63
Q

What are the causes of VUR

A

Primary - Abnoramlly short intravesicular portion of the distal ureter. Or secondary due to distal obstruction - posterior urethral valves or neurogenic bladder.

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64
Q

How many grades are there when assessing VUR.

A

5

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65
Q

What are the two radionuclide scans that can be performed for VUR ?

A
  1. Radionuclide cystogram: inserting Tc99m into the bladder, assessing for reflex.
  2. Tc-99m DMSA renal scintigraphy scan - gold standard for detection of renal cortical scarring (seen as areas of relative photopenia
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66
Q

What does the dropping Lilly sign indicate ?

A

Duplex kidney - reduced number of calycs
- duplicated system with a functioning lower pole calycs which is displaced inferiorly by the hydronephrotic upper pole.
Renal Ptosis - normal number of calcyes

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67
Q

What are the common causes of uteropelvic junction obstruction?

A

Abnormal development of the upper segment of the ureter
Aberrant renal artery
Aperistaltic segment of the ureter

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68
Q

How would prune belly syndrome and posterior urethral valves differ on imaging?

A

Prune belly - the entire urethral system is dilatated
Posterior urethral valves - only from the prostatic urethra upwards is dilated.

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69
Q

What are the three major findings in prune belly syndrome ?

A

Lack of anterior abdominal walls
Dilataiton of the renal system
Cryptorchidism (bilateral undescended testes)

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70
Q

What are patients with horeshoe kidney at risk of ?

A

Damage from the vertebral body - so can’t play certain sports
Increased risk for stones, infection and cancer - Wilms, TCC and renal carcinoid.

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71
Q

What syndrome is associated with horseshoe kidney?

A

Turner’s syndrome

72
Q

What gene mutation is seen in multilocular cystic nephroma ?

A

DICER 1 gene mutation ( this is not seen in the adult version )

73
Q

SImple renal cysts are found in which syndromes ? ?

A

Tuberous sclerosis
Von Hippel-lindau

74
Q

ADPKD is associated with extra-renal cysts where ?

A

The liver

75
Q

What is APRKD associated with ?

A

Hepatic fibrosis - it is inversely related to the severity of renal disease

76
Q

Causes of cystic renal lesions?

A

multilocular cystic nephroma
Multicystic dysplastic kidney
Simple renal cyst
ADPKD
ARPKD

77
Q

What does Wilms tumours arise from ?

A

Persistent Metaneprhic blastema (one of the 2 embryolical tissues that form the kidney )

78
Q

Which patients are screened for Wilms tumour

A

Beckwith-wiedemann syndrome
WAGR syndrome - Wilms tumour, aniridia, genitourinary anomliaise, mental retardatation
Horseshoe kidney
Trisomy 18

79
Q

What subtype of Wilms tumour would you expect with bone metastasis ?

A

Clear cell Wilms tumour

80
Q

What subtype of RCC affects sickle cell disease patients ?

A

Renal medullary carcinoma

81
Q

What age group do rhabdoid tumours of the kidney occur ?

A

< 1
Maybe < 2

82
Q

What is the cause of rhabdoid tumours of the kidney

A

Deletion of hte long arm Ch 22 - which is a tumour suppressor gene

83
Q

What are the most common primary malignancies that metastases to the kidneys ?

A

Neuroblastoma
Leukaemia
Lymphoma

84
Q

What age does Wilms tumour occur in ?

A

3-5 years

85
Q

How can you usually differentiate between Wilms tumour and mesoblastic nephroma

A

Age of patient
Mesoblastic nephroma - occurs in children < 1 yrs
Wilms tumour 3-5

However both are removed surgically as cannot determine between them

86
Q

Over what size are angiomyolipomas at risk of haemorrhage ?

A

Greater than 4cm

87
Q

What condition are Angiomyolipomas associated with ?

A

Tuberous sclerosis

88
Q

Are nephroblastomatosis usually uni or bilateral?

A

Bilateral, multi focal and confluent

89
Q

Whats the most common site for mets in Wilms tumour ?

A

Lung

90
Q

What is a differential list for multiple solid renal masses ?

A

Nephroblastomatosis
Lymphoma
Multi focal pyelonephritis
Multiple infarcts

91
Q

What is neuroblastoma

A

A malignancy that arises from primitive neural crest cells of the sympathetic nervous system.

92
Q

What is the most common extracranial malignancy in children ?

A

Neuroblastoma

93
Q

Which syndromes are neuroblastoma associated with

A

NF-1
DiGeorge
Hirschsprungs
Beckwith-Wiedemann

94
Q

What type of bone lesions are seen in neuroblastoma ?

A

Lytic and permeative

95
Q

If a neuroblastoma crosses beyond the contra lateral vertebral body what stage is it ?

A

Stage 3 - as it has crossed the midline

96
Q

What are the features of hand-Schiller-Cristian disease?

A

Diabetes insipidus
Exopthalmos
Skull lytic lesions

97
Q

What are the bad features of Ewing’s sarcoma ?

A

> 15
8cm
Metastases
No response to chemo
Raised LDH

98
Q

Where does Eosinophillic granulomas occur >

A

Long bones
Mandible
Spine - vertebral body collapse
Skull - punched out and sharply defined

99
Q

What type of peritosteal reaction does Eosinophilic granulomas have ?

A

Lamellated

100
Q

Why are bone scans not good for eosinophilic granulomas ?

A

Usually photopenic - so best reviewed on a skeletal survey

101
Q

How do people with langerhans cells histocytosis commonly present ?

A

Fever, pain and a mass

102
Q

What is langerhans cell histocytotiss ?

A

Uncontrolled monoclonal proliferation of langerhans cells and should be considered a malignancy

103
Q

What is a Brodies abscess?

A

A lytic, metaphyseal lesion seen in cases of infection. It usually extends to the growth plate.

104
Q

What is a sequestum

A

Dead bone

105
Q

What is the best test for investigating neuroblastoma ?

A

MIBG scan - 85-90% of patients will have upatake

106
Q

What type of bone lesion would you see in non-Hodgkin lymphoma ?

A

Osteolytic

107
Q

How many phases are used in a paediatric bone scan. ?

A

3 phases:
1) vascular phase 0-20seconds
2) blood pool phase
3) delayed phase

108
Q

What is the most common cause of osteomyeltisis in patients with sickle cell disease ?

A

Salmonella

109
Q

What radiographs would you obtain for suspected dysplasia ?

A

AP and lateral skull
Chest
DP of the LEFT hand (due to bone age)
AP leg - just one
AP arm - just one
AP abdo and pelvis
Lateral thoracolumbar spine

110
Q

When describing the areas involved for limb shortening :
Where is
1 . Acromelic
2. Mesomelic
3. Rhizomelic

A

Acro - distal hands or feet
Meso - middle (ulna/tibia )
Rhizo (proximal )

111
Q

What is Brucks syndrome ?

A

Osteogenesis imperfector with contractures

112
Q

What is osteopoikilosis ?

A

Sclerosing bony dysplasia with lots of bony islands

113
Q

What is classic shape of bones in achondroplasia ?

A

Trident hands

114
Q

What feature on a CXR would you seen in osteogenic imperfects ?

A

Ribbon ribs

115
Q

What is a gynmastic wrists ?

A

Widening of the physis secondary to excess stress

116
Q

What is the most common salter Harris fracture ?

A

Salter Harris 2 - 75%

117
Q

What is a tillaux fracture ?

A

A fracture through the anterolateral aspect of the distal tibia, salter Harris 3.
Various displacement.

118
Q

What is a toddlers fracture?

A

Undisplaced oblique fracture through the tibial metadiaphysis caused by rotational forces to the leg

119
Q

What views does a skeletal survey include ?

A

Frontal views of all long bones, rib views with obliques, skull, pelvis, hands/feet and entire spine.

120
Q

Which fractures are highly suspicious for child abuse ?

A

Posterior rib fracture
Scapula fracture
Sternum
Spinousus process
Classic metaphyseal lesion - bucket handle fracture

121
Q

What is a normal alpha angle ?

A

> 60

122
Q

When does femoral head ossification occur ?

A

6 months

123
Q

What is the most common cause of septic arthritis ?

A

S.aureus

124
Q

What is a systemic cause of bilateral perthes?

A

Avascular necrosis

125
Q

What would perthes show on a bone scan ?

A

Reduced uptake

126
Q

What would late perthes show on a bone scan ?

A

Increased uptake due to repair mechanisms

127
Q

Where is kleins line drawn and what does it show ?

A

Kleins line is drawn along the lateral margin of the femoral neck - if it doesn’t intersect the femoral head or if there is assymetry, it can indicate SUFE

128
Q

What age does SUFE affect ?

A

Boys 10-16 years

129
Q

How is SUFE treated ?

A

Screw fixation

130
Q

How many stages are there in a bone scan?

A

3 phases
1) early blood phase - vascularity
2) Blood pool phase - looking at soft tissue involvement
3) Delayed images - reflect the osteoblastic response to underlying disease

131
Q

What bug commonly causes osteomyeltisis in sickle cell patients ?

A

Salmonella

132
Q

Where does discitis commonly occur in children ?

A

Lumbar spine

133
Q

TORCH infections

A

Toxoplasmosis
Other - syphillis
Rubella
CMV
Herpes Symplex

134
Q

What is the Wimerger sign

A

Destructive erosion of the medial aspect of the proximal tibial metaphysics

135
Q

What is the wimberger ring sign ?

A

Scurvy - showing increased density of the ossification centres

136
Q

What infection can cause symmetric periosteal reaction in a child ?

A

Syphilis

137
Q

what are the common sites of ankylosis in juvenile rheumatoid arthritis

A

wrists, carpometacarpal joines and cervcal spine

138
Q

what is Klippel-Feil syndrome

A

Fusion of one or more cervical spine - associated with vertebral segmentation anomalise

139
Q

What is stills disease ?

A

Acute systemic subtype of JIA affecting children under the age of 5. They present with fever, anaemia, leukocytosis, hepatosplenomeagaly and polyarthiris

140
Q

is JIA usually RF positive or negative ?

A

Negative

141
Q

what is rhizomelia ?

A

proximal limb shortening - femur or humerus

142
Q

what is mesomelia ?

A

Middle limb shortening - radius/ular or tibia/fibular

143
Q

What is acromelia

A

Distal limb shortening - hand or foot,

144
Q

what is Amelia ?

A

Limb is absent

145
Q

what are the lumbosacral imaging features of achondroplasia ?

A

Narrowing of the interpedcuncular distances of the lower spine.
Flatenning of the acetabulum
Trident hand
Mickey Mouse ears / tombstone ileum

146
Q

what is the most common leathal skeletal dysplasia ?

A

Thanatophoric dysplasia

147
Q

what is the radiographic finding of cleidocranial dystosis ?

A

Lack of one or both of the clavicles.

148
Q

what is wormian bone ?

A

Lots of bones in the skull due to extrostosis

149
Q

which type of osteogenesis is lethal ?

A

type 2

150
Q

what are the classic clinical feature of osteogenesis imperfect ?

A

Blue sclera, bowing of the long bones. hearing impairment due to osteosclerosis. Wormain bones

151
Q

Causes of stippled epiphyses

A

Chondrodysplasia punctata
Multiple epiphyseal dysplasia
Hypothyroidism
Complication of mother warfarin use

152
Q

what’s the difference between mafucci syndrome and Ollier disease ?

A

mafucci syndrome is characterised with venous malformations which cause phleboliths . Maffucci has a high risk of malignant transformation

153
Q

what’s the difference between extosis or osteochondromatosis

A

osteochondromatosis have a cartilaginous cap

154
Q

What radiology sign might you seen in lead poisoning ?

A

Lead lines in the fibula

155
Q

What radiology sign might you seen in lead poisoning ?

A

Lead lines in the fibula

156
Q

What is hurlers syndrome ?

A

A mucopolysaccharide disease - lysosomal storage disease. This builds up and causes cell death

157
Q

What vertebral body abnormality might you see in hurlers syndrome

A

Anterior breaking of the vertebral bodies - inferiority

158
Q

What is rickets caused by.

A

Inadequate VItamin D

159
Q

What is rachitic rosary ?

A

Anterior cupping of the ribs with widening of the rib epiphyseal cartilage

160
Q

What MRI finding might you see in rickets ?

A

Loss of the black-white-black line corresponding to an in distinct ZPC

161
Q

What is oncogenic rickets ?

A

Tumour inducing phosphate depletion resulting in osteomalcia or rickets

162
Q

What might cause oncogenic rickets ?

A

Nonossifying fibroma or hemangiopericytoma ( rare tumour that grows in the soft tissues and contains all the different types of body structures e.g. fat, muscle, soft tissue, tendons etc)

163
Q

What is the most common primary bone tumour ?

A

Osteosarcoma

164
Q

Where is conventional osteosarcoma most commonly found ?

A

Around the knee, , distal femur or proximal tibia

165
Q

What is the second most common primary bone tumour ?

A

Ewings sarcoma

166
Q

What is ewings sarcoma ?

A

Aggressive tumour of small round blue cells - of the neuroectodermal differentiation

167
Q

What is the most common site of mets for ewings sarcoma ?

A

Lungs

168
Q

What is the most common paediatric malignancy ?

A

Leukaemia

169
Q

How often are bony changes seen in leukaemia ?

A

50% of cases

170
Q

What radiographic feature might you seen in leukaemia

A

Lucent metaphyseal bands

171
Q

What is Blount disease ?

A

Osteochondrosis of the proximal tibial metaphysis causing tibia virus and internal rotation eventually leading to progressive deformity, gait deviations and leg-length discrepancy

172
Q

Where does madelung deformity affect ?

A

Radius

173
Q

Where does panner disease affect ?

A

Capitellum

174
Q

What does prostaglandins cause to bones in newborns ?

A

Thought to slow osteoclast bone resorption leading to a periostea’s reaction

175
Q

what syndrome is associated with annual pancreas ?

A

downs synrome

176
Q

what is adrenoleukodystrophy?

A

a inherited disorder x-linked resulting in the build up of VLCFA (Very long chain fatty acids) which results in demylination. mainly affecting the optic and auditory pathway

177
Q

What is Carney’s triad ?

A

pulmonary hamartomas, gastric leiomyosarcomas and extra-adrenal paragangliomas