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Phase 3a > Paeds 4 - Endocrine, Malignancy, and MSK > Flashcards

Paeds 4 - Endocrine, Malignancy, and MSK Flashcards

1
Q

What is a preventable cause of learning difficulty?

A

Congenital hypothyroidism

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2
Q

What are the causes of congenital hypothyroidism?

A

Maldescent of thyroid
Dyshormonogenesis
Iodine deficiency

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3
Q

What are the common symptoms of congenital hypothyroidism?

A 
Failure to thrive
Constipation
PNJ
Pale, cold, mottled skin
Coarse facies and large tongue
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4
Q

What is the Guthrie test?

A

Neonatal blood spot screening between days 5-9 of life

Tests for CF, sickle cell, congenital hypothyroidism, etc

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5
Q

What is the treatment of hypothyroidism?

A

Thyroxine lifelong

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6
Q

What are the causes of hyperthyroidism in children?

A

Graves’ disease (autoimmune thyroiditis)

Neonatal - transplacental transfer of TSIs

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7
Q

What is the acronym for hyperthyroidism?

A 
Sweating
Weight loss
Emotionally labile
Appetite increase
Tremor and tachycardia
Intolerance to heat/irregular periods
Nervousness
Goitre and GI - diarrhoea

Eye signs (exophthalmos and lid lag) not common in children

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8
Q

How is hyperthyroidism diagnosed?

A

Increased T3 and T4

Very decreased TSH

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9
Q

How is hyperthyroidism treated?

A

Carbimzole/propythiouracil
Radioactive iodine
Thyroidectomy

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10
Q

Which hormone promotes bone formation via osteoblasts?

A

Parathyroid hormone

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11
Q

What occurs when calcium levels are low?

A

PTH promotes bone resorption via osteoclasts, increases renal uptake of calcium, activates metabolism of Vit D to increase gut Ca absorption

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12
Q

What are the symptoms of hypoparathyroidism?

A

Hypocalcaemia = muscle spasm, diarrhoea, stridor, fits

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13
Q

What are the symptoms of hyperparathyroidism?

A

Hypercalcaemia = constipation, polyuria, renal and gallstones, polydipsia, abdo pain, anorexia

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14
Q

What is the treatment of acute hypocalcaemia?

A

IV calcium gluconate

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15
Q

What is the treatment of acute hypercalcaemia?

A

Rehydration and bisphosphonates

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16
Q

What is the name of the condition where primary adrenal cortical insufficiency occurs?

A

Addison’s disease

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17
Q

What are the main causes of Addison’s disease?

A

Autoimmune

Haemorrhage

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18
Q

What is the acute presentation of Addison’s disease in infants?

A

Low sodium and glucose, high potassium
Dehydration and hypotension
Circulatory collapse

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19
Q

Older children with Addison’s disease present more subtly, with…

A

Vomiting
Brown hyperpigmentation
Growth failure
Fatigue

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20
Q

What hormone production is disrupted in Addison’s disease?

A

Decreased cortisol

Increased ACTH

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21
Q

What is the treatment of an adrenal crisis?

A

IV saline, glucose, hydrocortisone

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22
Q

What is the main treatment of Addison’s disease?

A

Long term mineralo-corticoid and glucocorticoid replacement

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23
Q

Glucocorticoid excess is known as?

A

Cushing syndrome

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24
Q

What conditions require long term steroids that can result in Cushing syndrome? (ACTH independent)

A

Nephrotic syndrome
Asthma
Severe bronchopulmonary dysplasia

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25
What are the symptoms of Cushing syndrome?
``` Growth failure Face and truncal obesity Red cheeks Hirsuitism and striae Bruising Hypertension Muscle wasting and weakness ```
26
How is Cushing syndrome diagnosed?
Loss of diurnal variation of cortisol (high in morning) | Dexamethasone has no effect on plasma cortisol levels
27
Give three causes of limp in a child
``` Osteomyelitis Fracture Perthes disease Septic arthritis Sickle cell crisis Slipped epiphysis ```
28
What are the likely organisms for MSK infections in infants, <4 years, and >4 years?
Infants - Group B strep, staph, coliforms <4 years - Staph, pneumococcus, haemophilus >4 years - Staph, gonococcus
29
What is a pathological cause of genu varum?
Bowed legs - ricketts
30
What is the name for knock knees?
Genu valgum
31
What is pes planus?
Flat feet
32
What is important to exclude in a child who toe-walks?
Duchenne muscular dystrophy JIA Spastic diplegia
33
Talipes equinovarus can be caused by what?
Oligohydramnios, IUGR
34
What is pes cavus?
High arched foot
35
Describe the spectrum of disorders in developmental dysplasia of the hip
Dysplasia to subluxation to complete dislocation
36
What are the risk factors for DDH?
Family history Breech Macrosomia/first born child Female sex x6
37
How is DDH diagnosed?
Routine neonatal screening Later - limp or abnormal gait Ultrasound
38
What is a complication of DDH?
Necrosis of femoral head
39
What is scoliosis?
Lateral curvature in the frontal plane of the spine
40
What is the most common cause of scoliosis?
Idiopathic
41
What is infection of the metaphysis of the long bones?
Osteomyelitis
42
What are the most common sites for osteomyelitis?
Distal femur and proximal tibia
43
What are the symptoms of osteomyelitis?
Pseudoparesis and pain in limb Acute febrile illness Swelling, erythema, tender Sterile effusion of adjacent joint
44
What is seen on X-Ray in osteomyelitis?
Soft tissue swelling and subperiosteal new bone formation after 7 days
45
How is osteomyelitis managed?
IV antibiotics | Aspiration or surgical decompression if atypical or immunocompromised
46
What are the complications of osteomyelitis?
Bone necrosis | Limb deformities
47
What is Osgood-Schlatter disease?
Osteochondritis of the patellar tendon insertion at the knee
48
What is important in examining a painful knee?
Examine the hip as hip pain is often referred to the knee
49
Give three red flags for back pain
``` Young age High fever Night waking Focal neurological signs Weight loss ```
50
Give three causes of back pain
Muscle spasm or injury Benign or malignant tumours Spinal cord compression Vertebral osteomyelitis or discitis
51
Avascular necrosis of the capital femoral epiphysis of the femoral head is known as...
Perthes' disease
52
What is seen on X-Ray in Perthes' disease?
Increased density in femoral head | Fragmented and irregular bone
53
Who typically gets Perthes' disease?
Boys aged 5-10 years
54
What are the symptoms of Perthes' disease?
Insidious hip or knee pain, limp | Pain increases with movement, especially internal hip rotation
55
How is Perthes' disease treated?
Bed rest and analgesia if under 6 | Calipes
56
What is Perthes' disease a risk factor for?
Degenerative arthritis in adult life
57
Transient synovitis is usually accompanied by...
A viral infection (can be recent)
58
What is inflamed in transient synovitis?
Synovium of the capsule of the hip joint usually
59
How does transient synovitis present?
Sudden onset of hip pain or limp No pain at rest Decreased range of movement, especially internal rotation Afebrile
60
What causes a slipped capital femoral epiphysis?
Fracture through the growth plate (physis) which results in slippage of the epiphysis Epiphysis is displaced posteroinferiorly
61
Who typically gets a SCFE?
Obese boys 10-15 years
62
How does SCFE present?
Waddling gait Limp or hip pain Decreased range of movement
63
How is SCFE managed?
X-Ray | Fixation surgery to prevent avascular necrosis
64
What does a septic joint look like?
Erythematous, swollen, warm, acutely tender joint, with decreased range of movement and pseudoparalysis in an unwell child
65
How is septic arthritis managed?
US guided aspiration of joint fluid to culture and identify organisms IV antibiotics
66
What is the name for brittle bone disease?
Osteogenesis imperfecta
67
Define juvenile idiopathic arthritis
Persistent joint swelling (>6 weeks) before 16 years, in the absence of infection or any other cause.
68
What are the main subtypes of JIA?
Oligoarthritis Polyarthritis (rheumatoid factor positive or negative) Systemic arthritis Psoriatic arthritis
69
What is gelling in JIA?
Stiffness after periods of rest e.g. in the morning
70
What are the other symptoms of JIA?
Pain Intermittent limp Swelling Deformities such as leg lengthening, valgus deformity, fever
71
What are the complications of JIA?
``` Anterior uveitis (can lead to visual impairment) Flexion contractures Growth failure Delayed puberty Osteoporosis ```
72
What is the use of NSAIDs for in JIA?
Relieve symptoms
73
What is the first line treatment for JIA?
Ultrasound guided steroid periarticular injections
74
What are the systemic symptoms in systemic JIA?
Evanescent salmon pink rash Lymph node enlargement Hepatomegaly/splenomegaly Serositis e.g. pleuritis, pericarditis
75
What is first line treatment if multiple joints are affected in JIA?
Methotrexate SC
76
Which other drugs may be used in JIA?
Etanercept (anti-TNF) Sulfasalazine Tocilizumab
77
Name an opportunistic infection in immunocompromised children
Pneumocystis jiroveci pneumonia Aspergillus or candidiasis VZV
78
Which is the most common childhood leukaemia?
Acute Lymphoblastic Leukaemia (80%)
79
Which syndrome has an increased risk of ALL?
Down syndrome
80
What are the main symptoms of ALL?
``` Fever Anaemia Infection Bruising Petechiae Hepatosplenomegaly Lymphadenopathy ```
81
What is seen on blood film in ALL?
Blast cells
82
Which chromosome is translocated in leukaemia?
Philadelphia (short arm of C22)
83
What is seen on FBC in leukaemia?
Low haemoglobin | Thrombocytopenia
84
What is the treatment of leukaemia?
Chemo - methotrexate, vincristine, thiopurine | Bone marrow transplant
85
What brain tumour is the most malignant in children?
Glioblastoma multiforme
86
What are the symptoms of increased ICP in children?
Headache worse in the morning and vomiting Papilledema Ataxia Visual changes
87
What are the symptoms of increased ICP in neonates?
Vomiting Tense fontanelle Torticollis Skull sutures separate
88
Which type of lymphoma is more common in childhood?
Non-Hodgkins' lymphoma
89
50% of NHL is due to...
EBV
90
How does lymphoma present?
Painless cervical lymphadenopathy
91
How is lymphoma investigated?
LN biopsy and then BM biopsy
92
What is neuroblastoma?
Tumour of the neural crest tissue found in the adrenal medulla or sympathetic chain
93
How does a neuroblastoma typically present?
Abdominal mass, weight loss, pallor, hepatomegaly
94
How is neuroblastoma diagnosed?
Increased urinary catecholamines | US and MRI
95
What is another name for nephroblastoma?
Wilms' tumour
96
What syndrome is associated with Wilm's tumour?
Denys-Drash
97
What are the symptoms of Wilm's tumour?
Large abdominal mass, anorexia, haematuria, hypertension
98
A soft tissue sarcoma in the head or neck will cause what symptoms?
Proptosis Nasal obstruction Blood stained nasal discharge
99
A soft tissue sarcoma in the GU tract will cause what symptoms?
Dysuria Urinary obstruction Blood stained vaginal discharge
100
What are the risk factors for osteosarcoma?
13q14 deletion Paget's disease Li-Fraumeni syndrome
101
What type of tumour is Ewing's sarcoma?
Malignant small, round, blue cell tumour
102
What is the main symptom of bone tumours?
Persistent bone pain
103
What are the risk factors for retinoblastoma?
80% family history - chromosome 13
104
How does retinoblastoma present?
Leukocoria - white pupillary reflex | StrabismusRed eye
105
How is retinoblastoma treated?
Enucleation of eye Chemotherapy Laser therapy Radiotherapy
106
What laboratory finding indicates germ cell tumour?
Increased alpha-FP and bHCG
107
What are the symptoms of type 1 diabetes?
``` Hyperglycaemia Ketonuria Polydipsia and polyuria Weight loss Metabolic acidosis with respiratory compensation ```
108
What is the management of DKA?
High flow oxygen Correct hypovolaemic shock with normal saline Commence IV insulin infusion Close monitoring of Serum glucose, Na, K, and cerebral oedema
109
What are the symptoms of DKA?
``` Vomiting and diarrhoea Abdominal pain Pear drop odour to breath Anorexia and fatigue Kussmaul respiration (deep sighing breathing) ```
110
What are the risk factors for DKA?
Infection | Poorly controlled diabetes
111
What laboratory findings would you see with metabolic acidosis?
pH<7.3, HCO3 <15
112
What would you find on urine dipstick in newly presenting diabetes?
Glucose and ketones
113
How does the breathing assessment differ in basic life support between adults and children?
In children you do 5 rescue breaths if the child is not breathing (not adults)
114
What is the required rate of chest compressions in paediatric life support?
100-120 a minute at 1/3 the depth of the chest
115
Give three possible causes of left hip pain in a 5 year old boy.
Transient synovitis Septic arthritis Perthes' disease Malignancy
116
What investigations are needed in a child with a limp?
X-Ray Bloods - FBC, CRP Joint aspirate if febrile
117
What is the treatment of bone marrow failure in ALL?
Granulocyte colony stimulating factor - stimulates bone marrow to produce RBC
118
What is the treatment of febrile neutropenia in ALL?
Tazocin/teicoplanin
119
What is tumour lysis syndrome?
Renail failure secondary to increased levels of urate, phosphate, and potassium at initiation of chemotherapy
120
What is the treatment of tumour lysis syndrome?
Hyperhydration Rasburicase Dialysis
121
Define neonate
A baby up to 28 days of age
122
What is the average age of diagnosis of retinoblastoma?
18 months
123
What is the further management of breech babies if their newborn examination is normal?
Ultrasound at 6 weeks to check for DDH (regardless of mode of delivery)
124
What are the characteristics of pain in Osgood Schlatter disease?
``` Located to the tibial tuberosity Usually unilateral Gradual and intermittent in onset, but can progress to be severe and continuous Relieved by rest Made worse by kneeling and jumping ```
125
What is the inheritance pattern in achondroplasia?
Autosomal dominant | But 70% are sporadic
126
What are the signs of achondroplasia?
Macrocephaly with frontal bossing Trident hand Lumbar lordosis Short limbs and fingers

Phase 3a (18 decks)

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