Paediatrics Flashcards
1
Q
What is a comitant deviation?
A
A deviation that is the same magnitude regardless of gaze position and is present in all directions of gaze.
2
Q
What is an incomitant deviation?
A
A deviation in which the magnitude changes depending on the direction of gaze or is only present in certain directions of gaze.
3
Q
What is the management for strabismus?
A
- Conservative: glasses, prisms, orthoptic exercises to improve control over eye muscles.
- Surgery: resection and recession.
Botox injections paralyse the muscle that is pulling the eye in a certain direction.
4
Q
What is the management for amblyopia?
A
- Glasses to correct refractive error.
- Patching of good eye.
- Atropine eye drops (antimuscinaric so dilate) in better seeing eye to dilate the pupil.
5
Q
What is the diagnostic investigation for Hirschsprung’s disease?
A
Suction rectal biopsy.
6
Q
What part of the bowel is Hirschsprung’s disease normally found in?
A
75% refined to the recto-sigmoid.
7
Q
What electrolyte disturbance is found in pyloric stenosis?
A
Hypokalaemic, hypocholoraemic, metabolic alkalosis.
8
Q
Where is the mass found in intussusception?
A
RLQ.
9
Q
What is the diagnostic investigation for intussusception?
A
USS: shows a target/doughnut sign.
10
Q
What is the gold standard investigation for appendicitis?
A
CT abdomen.
11
Q
What is the investigation for Meckel’s diverticulum?
A
Technetium scan.
12
Q
What is the diagnostic investigation for malrotation?
A
Upper GI contrast study.
13
Q
What procedure is performed in malrotation?
A
Ladd’s procedure: rotates bowel anti-clockwise
1. Small bowel on the right
2. Large bowel on the left
3. Appendicectomy.
14
Q
What is the investigation for necrotising enterocolitis?
A
X-ray abdomen:
Distended loops of bowel
Thickening of bowel wall with intramural gas
Rigler and football sign.
15
Q
What is mesenteric adenitis typically associated with?
A
URTI with cervical lymphadenopathy.
16
Q
What is the treatment for abdominal migraine?
A
Pizotifen (serotonin receptor antagonist). Used as prophylaxis in children with frequent, severe symptoms.
17
Q
What is the gold standard investigation for Coeliac disease?
A
Intestinal (jejunal) biopsy showing villous atrophy and crypt hyperplasia.
18
Q
What area of the digestive tract does Crohn’s affect?
A
Mouth to anus.
19
Q
What are the features of Crohn’s?
A
Transmural inflammation.
Discontinuous with skip lesions.
Granulomatous (look for granulomas on histology)
Rectal sparing.
Fissures, fistulae, abscesses and strictures.
Perianal disease.
20
Q
What are the extra GI symptoms of Crohn’s?
A
Fever, arthritis, uveitis.
21
Q
What is an investigation for Crohn’s?
A
Endoscopy.
22
Q
What is the management for Crohn’s?
A
Exclusive enteral nutrition (feed them loads of milk for 7-8 weeks and they get better).
Corticosteroids: prednisolone, budesonide.
Aminosalicylates: sulfasalazine, mesalazine.
Antibiotics.
Immunomodulators: methotrexate, azathioprine, 6-mecaptopurine.
Biologics: infliximab, adalimumab.
Surgery.
23
Q
What are the features of UC?
A
Affects only the mucosa.
No granulomas.
No skip lesions (the whole colon is affected).
Ulcers.
24
Q
What are the extra GI symptoms of UC?
A
Mouth ulcers, arthritis, erythema nodosum, spondylitis.
25
What is the management for UC?
Mild attacks: topical steroids when confined to rectum/sigmoid colon, sulfasalazine for more extensive disease.
Severe disease: systemic steroids.
Colectomy in 1/3 patients which is curative.
26
What does blood in vomit suggest?
Oesophagitis, peptic ulcer, oral/nasal bleeding, malrotation.
27
What is the gold standard investigation in GORD?
Gold standard: pH study.
* Probe into child’s nostril and down oesophagus.
* X-ray to check it is in the right place
* Eat and drink as normal and probe measures pH
* Worn for 24 hours.
28
What is a complication of diarrhoea?
Hypernatraemia dehydration: water exceeds sodium loss as water shifts from intracellular to extracellular.
Symptoms: jittery movements, increased muscle tone, hyperreflexia, convulsion, drowsiness, doughy skin, irritable.
29
What is a contributing factor to Toddler's diarrhoea?
Poor diet: fat, fluid, fruit juices, fibre.
30
What is an osmotic laxative?
Movicol (macrogol), lactulose.
31
What is a bulking laxative?
Fybogel.
32
What is the management of Wilm's tumour?
* Chemotherapy for 6 months to shrink.
* Nephrectomy: full if only one kidney, partial if both kidneys affected
* Radiotherapy
* 80% cure.
33
How do you treat H. Pylori?
Triple therapy: PPI, clarithromycin, amoxicillin/metronidazole.
34
What pancreatic enzymes are absence in CF?
Lipase, proteases and amylase. Must be replaced for every meal.
35
What happens to the abdominal wall in congenital diaphragmatic hernia?
Abdominal wall concaves/scaphoid abdominal wall.
36
What is the management of congenital diaphragmatic hernia?
Intubation and ventilation.
37
What is the management of an inguinal hernia?
Urgent surgical referral for surgery within 2 weeks.
38
What murmur is heard in ToF?
Ejection systolic murmur at left sternal edge (pulmonary area).
39
When is the corrective surgery for ToF?
6-9 months old.
40
What are the stages of surgery for ToGV?
* Balloon atrial septostomy: creates a hole between the 2 atria to allow mixing
* Arterial switch procedure: the aorta and PA are transected above the valves and switched over. Done electively later in life, not as an emergency straight after birth.
41
What is the management for a complete AVSD?
Medical treatment for heart failure.
Surgical repair at 3-6 months.
42
What is the murmur in VSD?
Pansystolic murmur in left lower sternal edge.
43
What is the murmur in ASD?
Ejection systolic murmur left upper sternal edge.
44
What are the signs of an innocent murmur?
4 S’s:
1. Soft
2. Systolic
3. aSymptomatic
4. L Sternal edge.
45
What is the murmur in aortic stenosis?
Ejection systolic murmur at upper right sternal edge.
Carotid thrill.
46
What is the murmur in pulmonary stenosis?
Ejection systolic at upper left sternal edge.
Palpable thrill.
47
What heart condition is associated with Turner's syndrome?
Coarctation of aorta.
48
Where does the narrowing of the aorta in coarctation of the aorta typically occur?
At PDA.
49
What are the symptoms of coarctation of the aorta?
Become more severe with age. Firstly asymptomatic.
* SOB
* Arterial hypertension
* Intermittent claudication.
Circulatory collapse at 2 years old.
50
What are the signs for coarctation of the aorta?
Ejection systolic murmur at upper left sternal edge.
Radial: radial/radial: femoral delay.
Absent/weak femoral pulses.
51
What is the most common type of cardiomyopathy seen in children?
Dilated.
52
What is the management of dilated cardiomyopathy?
Symptomatic treatment: diuretics, ACE-I, beta blockers (treating the symptoms of heart failure).
Usually resolves spontaneously.
Some children may need a heart transplant.
53
What is the most common cause of heart failure in children?
Large VSD.
Causes right sided heart failure.
54
What are the signs that heart failure is right-sided?
Peripheral oedema.
Liver enlargement.
55
What are the signs that heart failure is left-sided?
Increase in HR, RR and crepitations.
56
What is the management of tricuspid atresia?
Shunting between the subclavian and pulmonary artery.
57
How does prematurity cause anaemia?
Not fully developed so insufficient erythropoietin production. Protein content of breastmilk not sufficient enough for haemopoiesis in premature infants.
58
What indicates haemolysis on bloods?
A high circulating reticulocyte count.
59
What is the management of sickle cell anaemia?
Antibiotic prophylaxis: twice daily penicillin against pneumococcus.
Acute crises: analgesia, hydration, oxygen.
Exchange transfusion: acute chest, stroke, priapism.
Chronic problems: hydroxyurea, hydroxycarbamide (increase the level of foetal haemoglobin which replaces the sickle haemoglobin).
60
What is a complication of sickle cell disease and parvovirus B19?
Aplastic crisis.
61
What is aplastic anaemia?
Bone marrow failure so the bone marrow is not producing any blood cells.
Reduction or absence in platelets, WBC, RBC in the bone marrow leading to peripheral pancytopenia.
62
What are the vitamin K dependant clotting factors?
10, 9, 7 and 2 (1972).
63
What deficiency is Haemophilia A?
Factor 8 deficiency.
64
What deficiency is Haemophilia B?
Factor 9 deficiency.
65
What factors does PT measure?
Factors 2, 5, 7, 10.
66
How does Von-Willebrand's disease present?
Presents with mucosal bleeding e.g. epistaxis, gum bleeds, menorrhagia or excessive bleeding after surgery.
Different to big bleeds in haemophilia.
67
What is the method of inheritance for haemophilia?
X-linked recessive.
68
What is Marfan's?
A connective tissue disorder caused by fibrillin deficiency.
69
What is the complication of Marfan's?
Regular echocardiograms to check for dilatation of the aorta so it aortic dissection can be prevented.
70
What does Ehler-Danlos affect?
Elastin defect so affects collagen.
71
What is Steven-Johnson syndrome?
* Flu-like symptoms initially
* Red/purple target-like rash that spreads and forms blisters
* Affected skin peels and dies off
* Mucous membranes of the mouth, throat, eyes and genital tract may also become blistered and ulcerated.
72
What drugs cause Steven-Johnson syndrome?
* Allopurinol
* Lamotrigine
* Penicillin
* Phenytoin.
73
What can cause necrotising fasciitis?
Chickenpox + NSAIDs.
74
What reduces cavernous haemangioma/"strawberry marks"?
Propranolol.
75
What are naevi associated with?
Turner's syndrome.
76
How many cafe-au-lait spots are needed for a diagnosis of neurofibromatosis?
>5.
77
What is the common cause of ancathosis nigricans?
Obesity.
Insulin resistance.
78
Where do branchial cysts typically present?
Not in the midline, tend to appear along the border of the sternocleidomastoid.
79
Where do dermoid cysts typically present?
Lateral aspect of the eye, produce sebaceous material. Beware because can communicate intracranially and cause meningitis.
80
What is rebound hyperglycaemia/Dawn phenomena?
Taking insulin before bed can lower glucose too much that it can trigger a release of hormones that send blood sugar levels high into a rebound.
81
How is mild/moderate hypoglycaemia managed?
1. Check BM to confirm.
2. Glucose tablets/glucogel/3 jelly babies. Wait 10 minutes and if no improvement, repeat.
3. Follow up with longer acting carbohydrate e.g. sandwich.
4. Check BMS again after 15 minutes.
82
How is severe hypoglycaemia managed?
1. Do not attempt to give anything by mouth
2. Glucagon: SC or IM injection (0.5mg if < 5 years, 1mg if > 5 years)
3. Wait 10 mins
4. When conscious give longer acting carbohydrate.
83
What level of blood sugar is hypoglycaemia?
<3.5
84
At what blood sugar level do cognitive symptoms begin?
<3.0
85
What type of breathing is seen in DKA?
Kussmaul: hyperventilation due to acidosis.
86
How is DKA managed?
1. Aggressive fluid resuscitation first (0.9% NaCl) IV if not alert, PO if alert.
2. Insulin SC (0.1 units/kg/hour) after fluids have been running for an hour.
87
What happens if a child is rehydrated too quickly in DKA?
Cerebral oedema.
88
What is the function of insulin?
Lowers blood glucose by stimulating uptake from the blood into muscle, kidney and fat cells.
Targets liver to convert glucose into glycogen.
89
What fasting plasma glucose is normal?
3.5-5.6mmol/l
90
What fasting plasma glucose is pre-diabetic?
5.6-7.0mmol/l
91
What fasting plasma glucose is diabetic?
>=7.0mmol/l
92
What post-prandial glucose is normal?
<7.8mmol/l
93
What post OGTT glucose is pre-diabetic?
7.8-11.0
94
What random plasma glucose is diabetic?
>=11.1
95
What Hba1c is normal?
4-5.6%
96
What Hba1c is pre-diabetic?
5.7-6.4%
97
What Hba1c is diabetic?
>6.5%
98
What are the sick day rules for T1DM?
* Never omit insulin
* 1-2 hourly BM measurements through night
* Check ketones regularly e.g. 3-4 hours through night
* Normal meal pattern even if appetite is reduced
* Fluids
* Continue to monitor glucose until it returns to normal.
99
What is the most common type of hyperthyroidism?
Grave's disease.
100
How is hyperthyroidism managed?
Carbimazole.
Propylthiouracil.
Either by a dose titration or block and replace with thyroxine.
101
What is the most common cause of hypothyroidism?
Autoimmune thyroiditis (Hashimoto’s).
102
What is the main cause of congenital hypothyroidism worldwide?
Iodine deficiency
103
What is the main cause of congenital hypothyroidism in the UK?
Maldescent of thyroid/absent thyroid.
104
What is the main cause of hypothyroidism in consanguineous families?
Dyshormonogenesis (inborn error of thyroid hormone synthesis).
105
What chromosome is affected by CF?
Chromosome 7.
106
How does CF present in a newborn?
Meconium ileus.
Prolonged jaundice.
107
What is found on a chest examination in CF?
* Hyperinflation of the chest due to air trapping
* Coarse inspiratory crepitations
* And/or expiratory wheeze
* Finger clubbing with established disease.
108
What is the gold standard test for CF?
Sweat test: excessive Na and Cl.
109
What is the management for CF?
* Chest physio 2x daily
* Physical exercise encouraged
* Continuous prophylactic oral antibiotics, usually flucloxacillin
* Rescue antibiotics when needed
* Nebulised Dnase: decreases sputum viscosity to increase clearance
* Oxygen and CPAP may be needed long term
* Lung transplant.
110
What nutrition is required in CF?
* High calorie and high intake diet essential: 150% of normal
* Oral enteric coated pancreatic replacement therapy with all meals and snacks.
111
What bacteria are CF patients particularly susceptible to?
* Staph aureus
* Haemophilus influenzae
* Pseudomonas aeruginosa.
112
What type of inheritance is seen in CF?
Autosomal recessive.
113
How is a moderate asthma attack defined?
* Sats >92%
* PEF >50%
* No clinical features.
114
How is a severe asthma attack defined?
* SpO2 < 92%
* PEF 33-50%
* Too breathless to talk or feed
* Heart rate - >125 (>5 years), >140 (1-5 years)
* Resp rate > 30/min (> 5 years), > 40 (1-5 years)
* Use of accessory neck muscles.
115
How is a life threatening asthma attack defined?
* SpO2 < 92%
* PEF < 33%
* Silent chest
* Poor respiratory effort (Normal PCO2).
* Agitation
* Altered consciousness
* Cyanosis.
116
How much does FEV1 improve by after a bronchodilator in asthma?
12%
117
What ICS is used in children <5?
Moderate dose.
118
What ICS is used in children >5?
Low dose.
119
How is an asthma attack managed?
SABA + 3 days prednisolone.
120
How is an acute asthma attack managed?
1. High flow oxygen
2. Nebulised or inhaled beta 2 agonist (salbutamol)
3. IV steroid (prednisolone or hydrocortisone)
4. Atrovent nebuliser (ipratropium bromide)
5. IV salbutamol bolus
6. Magnesium sulphate/aminophylline/salbutamol infusion.
121
What are the long-term risks of taking ICS?
* Adrenal suppression
* Growth suppression
* Osteoporosis
* Diabetes
* Cataracts.
122
What is the inheritance of a retinoblastoma?
Autosomal dominant.
123
Where is the retinoblastoma susceptibility gene?
Chromosome 13.
124
What cells are found on film in Hodgkin's lymphoma?
Reed-Sternberg.
125
What is the diagnostic investigation for Hodgkin's lymphoma?
Lymph node biopsy.
126
What are the investigations for Non-hodgkin's lymphoma>
Stage with CT/MRI.
Bone marrow aspirate.
CSF sample.
127
What is the benign version of a neuroblastoma?
Ganglioneuroma.
128
What do you think of with a miserable child and a big abdomen?
Neuroblastoma.
129
When is an LP contraindicated?
Raised ICP.
130
What syndrome is ALL associated with?
Down's syndrome.
131
How does ALL present?
Symptoms of pancytopenia:
* Infections (due to neutropenia)
* Lethargy and pallor (due to anaemia) + soft systolic murmur
* Abnormal bruising, petechiae, nose bleeds (due to pancytopenia).
Other:
* Malaise
* Fever
* Testicular swelling
* Anorexia
* Hepatosplenomegaly
* Lymphadenopathy (lumps and bumps)
* Bone pain (due to bone marrow infiltration).
132
What is the gold standard investigation for ALL?
Bone marrow aspirate.
133
What does a blood test in ALL show?
* Low Hb (anaemia)
* Thrombocytopenia
* Evidence of circulating blast cells
* WCC may be up or down
* Neutropenia.
134
What is the management for ALL?
Vincristine chemotherapy plus a steroid such as dexamethasone.
135
What are good prognostic features for ALL?
* Age 2-10
* Female
* WCC <50
* No CNS disease.
136
What are poor prognostic features for ALL?
* Age <2 or >10
* WCC >20 at diagnosis
* T or B cell surface markers
* Non-Caucasian
* Male.
137
What is inflamed in Osgood-schlatters
Tibial tuberosity.
138
What investigation is needed in torticollis?
C spine plain X-ray.
139
What is the test for a meniscal tear?
McMurray's test positive.
140
What is a toddler's fracture?
Spiral or oblique undisplaced fracture of the distal shaft of the tibia with an intact fibula.
141
What sign is positive in DDH?
Galleaze sign: child is laying down on the bed with their feet flat against the bed. Positive is when knees are different heights
142
What is the difference in presentation between septic arthritis and osteomyelitis?
In osteomyelitis, some passive movement is preserved.
143
What antibiotic is given in septic arthritis?
Flucloxacillin as usually Staph Aureus.
144
What criteria is used in septic arthritis?
Kocher's:
1. Fever > 38.5
2. Inability to weight bear
3. CRP >20
4. WBC >12.
145
How is a slipped upper femoral epiphysis managed?
Surgical emergency: in situ fixation with a cannulated screw.
146
What rotation is lost in slipped upper femoral epiphysis?
Internal rotation.
147
What is needed for JIA diagnosis?
Onset before 16th birthday.
No identified underlying cause.
Persistent joint swelling/inflammation.
Lasting at least 6 weeks (i.e. not reactive arthritis).
148
What are the X-ray features of JIA?
* Loss of joint space
* Joint deformity
* Soft tissue swelling
* Periarticular osteopenia.
149
What is the management for oligarticular JIA?
Inject the affected joint with steroid.
150
What is the management for polyarticular JIA?
System therapy (methotrexate is the safest and best option, not steroids in kids). Given as weekly dose and requires blood monitoring for liver function and bone marrow suppression.
If no response after 3 months, start biologic e.g. infliximab, adalimumab, Anti-TNFa.
151
What are the types of osteogenesis imperfecta?
* Type 1: mild.
* Type 2: fatal. Child dies early because the chest is too small to allow breathing. They have a lot of rib fractures, sometimes cause by the birth process, and their lungs don’t function at all.
* Type 3: progressively deforming, severe. Wormian bones at 1 year old.
* Type 4: moderate.
152
What is the inheritance pattern in osteogenesis imperfecta?
* Autosomal dominant in 85-90%
* Defects in type 1 collagen genes (the type of collagen in bones).
153
What is a sign of osteogenesis imperfecta?
Blue tinted sclera.
154
What shows on X-ray for osteogenesis imperfecta?
* Wormian bones (wiggly black lines in the skull): feels like bubble wrap under your fingers if you feel their skull
* Bent bones e.g. bent tibia
* Bowing of the femur
* Complete chest collapse in type 2 OI -> fatal .
155
How is OI managed?
Bisphosphonates: pamidronate. Prefer to give IV.
156
What is the recommended daily intake of Vitamin D?
400mg daily.
157
What biochemistry is shown in Rickett's?
Serum calcium levels low
Raised PTH levels
Low alkaline phosphate
Low phosphate.
158
What does X-ray show in Rickett's?
Bowed legs, splayed metaphyses, frayed metaphyses.
159
What are signs of a temporal lobe seizure?
* Strange warning feelings/aura: smell, taste, visual, ‘rising sensation’
* Automatisms: lip smacking, plucking clothing, pacing
* De ja vu and jamais vu
* Impaired consciousness: longer duration than a typical absence seizure.
160
What anti-epileptic should be avoided in absence and myoclonic seizures?
Carbamazepine.
161
What spikes are shown on benign rolandic epilepsy EEG?
Centro-temporal spikes on EEG.
162
What are the three types of spastic CP?
Hemiplegic.
Quadriplegic.
Diplegic.
163
What is affected in spastic CP?
UMN pathways affected in the periventricular white matter (pyramidal or corticospinal).
164
What is affected in ataxic CP?
Cerebellum affected.
165
What is affected in dyskinetic CP?
Basal ganglia and substantia nigra are affected (extra-pyramidal).
166
What are the symptoms of hemiplegic spastic?
* They will have one “bad side”
* Unilateral involvement of arm and leg
* Arm usually worse
* Face spared
* Tip-toe walking
* Fisting and flexion of the affected arm.
167
What are the symptoms of quadriplegic spastic?
* All 4 limbs affected
* Arms usually worse
* Trunk involved: extensor posturing, poor head control, low central tone
* Seizures
* Microcephaly
* Learning difficulties.
168
What are the symptoms of diplegia spastic CP?
* All 4 limbs affected
* But legs worse so arm function appears relatively normal
* Walking is abnormal.
169
What are the symptoms of dyskinetic CP?
* Athetoid movements: slow, involuntary, writhing movements, dystonia, choreoathetosis
* Oro-motor problems.
170
What are the symptoms of ataxic CP?
* Symmetrical
* Early trunk and limb hypotonia
* Poor balance
* Delayed motor development.
171
What are the signs in spastic CP?
* Increased limb tone (spastic)
* Brisk deep tendon reflexes
* Extensor plantar response (positive Babinski sign)
* Increased tone may suddenly yield under pressure (clasp knife).
172
What are the signs in dyskinetic CP?
* Fluctuating tone
* Involuntary movements
* Chorea, dystonia
* Intellect unimpaired.
173
What is used for spasticity in CP?
Baclofen or oral diazepam.
Botox.
