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Phase 3A 2021 > Paeds new > Flashcards

Paeds new Flashcards

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1
Q

Pneumonia defintion?

A
  • inflammation of the lung parenchyma (the part of lungs involved in gas transfer e.g. alveoli + resp bronchioles)
  • congestion caused by viruses or bacteria or irritants
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2
Q

Causes of pneumonia in children?

A

Viral more common

Viral: RSV, adenovirus, rhinovirus, influenza

Bacterial: Strepmpneumoniae, Hib

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3
Q

Presentation of pneumonia in children

A
  • temperature (over 38.5)
  • rapid breathing/difficulty breathing
    cough
  • chest pain
  • vomiting
  • decreased activity
  • loss of appetite/poor feeding
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4
Q

Investigations for paediatric pneumonia?

A
  • Sputum sample (can be difficult)
  • Blood cultures.
  • CXR: look for consolidation.
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5
Q

Describe the treatment of pneumonia.

A

PO amoxicillin.
Co-amoxiclav if complicated or unresponsive.

O2, analgesia, IV fluids if indicated.

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6
Q

Bronchiolitis:

  • causative agent?
  • age?
A
  • RSV
  • Babies 0-2
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7
Q

Investigations for bronchiolitis?

A

Nasopharyngeal aspirate or throat swab
RSV rapid testing and viral cultures
FBC

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8
Q

Home or hospital for bronchiolitis?

A

Hospital if severe apnoea/resp distress: grunting, RR>70, central cyanosis, sats<92%,

Consider hospital if rr>70, inadequate fluids, clinical dehydration

No role for antibiotics, steroids or bronchodilators

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9
Q

What is asthma?

A

Chronic obstructive resp disease characterised by episodic exacerbations of bronchoconstriction

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10
Q

Presentation of asthma?

A
  • Cough - nocturnal
  • SOB
  • wheeze/whistling
  • chest congestion/tightness
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11
Q

Diagnosis of asthma in children?

A
  • If under 3: may use wait and see approach
  • If under 5: need to go off the history
  • If over 5: spirometry, PEF
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12
Q

Acute management of asthma?

A
  • Oxygen if needed
  • SABA
  • Prendisolone 1mg/kg IV

If no improvement:

  • IV salbutamol bolus
  • Aminophylline/MgSO4/salbutamol infusion
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13
Q

Long-term asthma management in under 5s?

A
  1. SABA for wheeze episodes (salbutamol prn)
    • ICS (beclametasone)
    • Leukotrine-receptor agonist (montelukast)
  4. stop LTRA and refer to asthma specialist
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14
Q

List three possible side effects of inhaled corticosteroids

A
  • Adrenal suppression
  • Growth suppression
  • Osteoporosis (although this has not been shown to affect bone fractures)
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15
Q

Wheeze vs stridor?

A

Wheeze = expiratory, polyphonic

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16
Q

Inhalers: name 2 ‘preventers’.

A

ICS act as ‘preventers’ e.g. beclamethasone, budenoside.

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17
Q

Inhalers: name 2 ‘relievers’.

A

Beta agonists e.g. salbutamol.
Muscarinic antagonists e.g. ipratropium bromide.

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18
Q

Why might asthma treatment fail in children?

A
  1. Adherence.
  2. Wrong diagnosis.
  3. Environmental factors.
  4. Choice of drug.
  5. Bad disease.
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19
Q

Non-resp causes of wheeze?

A
  1. GORD
  2. bronchomalacia
  3. cystic fibrosis
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20
Q

What constitutes the upper airway?

A

Nose, pharynx, larynx

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21
Q

Name 3 URTI.

A
  1. Rhinitis.
  2. Otitis media.
  3. Pharyngitis.
  4. Tonsillitis.
  5. Laryngitis.
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22
Q

Name 3 LRTI.

A
  1. Bronchitis.
  2. Croup.
  3. Epiglottitis (bacterial).
  4. Tracheitis.
  5. Bronchiolitis.
  6. Pneumonia.
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23
Q

Would you expect a patient with bronchitis or with bronchiolitis to be hypoxic and tachypnoeic? Explain why.

A

Bronchiolitis.

Bronchiolitis affects the respiratory portion of the airway, where gas exchange takes place therefore you may see hypoxia and tachypnoea.

Bronchitis affects the conducting portion of the airway and so is unlikely to have these effects.

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24
Q

Name 4 LRTI that could be caused by RSV.

A
  1. Acute bronchiolitis.
  2. Wheezy bronchitis.
  3. Asthma exacerbation.
  4. Pneumonia.
  5. Croup.
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25
Why are infants more susceptible to descending infection?
Infants have a poor innate immune response and so are more susceptible to descending infections.
26
What is croup?
Acute larngotracheobronchitis.
27
Describe the cough that is associated with croup.
A barking seal like cough - often worse at night.
28
How do you treat croup?
usually self-limiting can give steroids e.g. dexamethosone, beclamethasone.
29
Describe the aetiology of recurrent wheeze.
1. Persistent infantile wheeze. 2. Viral episodic wheeze. 3. Asthma.
30
What is persistent infantile wheeze normally associated with/exacerbated by?
Persistent infantile wheeze tends to affect the small airways. It is associated with parental smoking or post-viral infection.
31
Are inhalers likely to help a child with persistent infantile wheeze?
No. Inhalers are unlikely to help; symptoms will improve as the child gets older.
32
Are inhalers likely to help a child with viral episodic wheeze?
Bronchodilators may help but there is no benefit from inhaled steroids. Symptoms are likely to improve with age.
33
Viral induced wheeze: * common causes? * affects which age?
* RSV, rhino virus * children aged 2-5
34
VIW vs bronchiolitis?
Bronchiolitis: babies, 3-5 days gradual build up VIW: 2-5 yrs, viral prodrome for 1-2 days then suddenly worsens
35
How to manage a viral induced wheeze?
Reliever inhaler for the minority=SABA=salbutamol If SABA 2-10 puffs PRN to max 4 hourly via a spacer Otherwise, supportive/comfort care
36
How to identify viral induced wheeze (how does it differ from asthma)?
* No wheeze with exercise * No interval symptoms * No excess of atopy * No benefit from regular inhaled steroids * No wheeze except with viral infections
37
Pathaphysiology of cystic fibrosis
Autosomal recessive Reduced airway surface liquid impedes mucus clearance allowing excessive bacterial growth Pancreas: duct occluded in utero leading to pancreatic insufficiency (can lead to CF-related DM) GI: increased mucus can cause meconium ileus Biliary tree: can have cholestasis leading to neonatal jaundice
38
Neonatal test to identify CF
Heel prick- Guthrie test
39
Investigations for CF:
Sweat chloride over 60mmol/L CXR, sweat test, glucose tolerance test, microbiology, LFT, coag, bone profile, PFTs- all generally involved in annual assessments
40
Name 2 respiratory illnesses that can present with stridor.
1. Croup - often a louder stridor. 2. Acute epiglottitis - often a quieter stridor due to inflamed epiglottis blocking oesophagus and trachea.
41
Why are children more at risk of epiglottitis than adults?
Epiglottis is floppier, broader, longer and angled more obliquely to trachea and have a larger tongue Therefore higher risk of acute airway obstruction
42
Aetiology of epiglottitis?
Reduced since the Hib vaccination Normally Haemophilus infleunzae and Streptococcus pneumoniae which locally invade V rarely due to trauma or non-infectious causes
43
Presentation of epiglottitis
1. S.O.B 2. Drooling 3. Difficulty swallowing (dysphagia) 4. Muffled voice (dysphonia) 5. Typically no cough 6. Stridor is a late sign
44
Describe the immediate management for acute epiglottitis.
Secure the airway - anaesthetist, ENT surgeon.
45
Management of epiglottitis
Oxygen, nebulised adrenaline, IV antibiotics (3rd gen cephalosporins eg ceftriaxone), IV steroids, nil by mouth until airway improved
46
Name a bacteria that causes acute epiglottitis.
H.influenzae B. Acute epiglottitis is a severe acute illness.
47
You do a lumbar puncture and find raised proteins and low glucose. Is this likely to be due to a bacterial or a viral infection?
Bacterial.
48
Give 3 potential consequences of hearing loss.
1. Speech and language delay. 2. Social problems e.g. behavioural issues. 3. Academic underachievement.
49
How does hearing loss in children often present?
1. Parental concern. 2. Speech, behavioural or educational problems. 3. Incidentally on screening.
50
What are the 3 types of hearing loss?
1. Conductive hearing loss. 2. Sensori-neural hearing loss. 3. Mixed.
51
Give 3 causes of conductive hearing loss.
1. Glue ear. 2. Ear wax. 3. Otitis media. 4. Perforated ear drum.
52
Describe the management of conductive hearing loss.
Conductive hearing loss is usually ENT managed: - Wait and wait - most will resolve on their own. - Grommet insertion. - Temporary hearing aid.
53
Give 3 risk factors for sensori-neural hearing loss.
1. Family history. 2. SCBU. 3. Consanguinity.
54
Describe the management of sensori-neural hearing loss.
Sensori-neural hearing loss is often managed by a paediatrician. Treatments involve hearing aids or cochlea implants.
55
How would you manage mixed hearing loss?
You would address the conductive problem first and then offer a hearing aid.
56
When is hearing tested in children?
1. New-born hearing screen. 2. School entry hearing test. 3. Long term monitoring is done in high risk groups.
57
Is the new-born hearing screen an objective or subjective test?
It is an objective test - response or no response. If there are concerns, the patient is followed up with evoked response audiometry.
58
What are the 3 aims of hearing testing in children?
1. Measure hearing threshold (dB). 2. To be frequency specific (Hz). 3. Obtain single ear information if possible.
59
Name 4 types of subjective hearing testing.
1. Behavioural observational audiometry. 2. Distraction testing. 3. Visual reinforcement audiometry. 4. Performance testing and play audiometry.
60
Name 2 bacterial and 2 viral organisms that can cause acute otitis media.
Bacterial: S.pneumoniae., H.influenzae. Viral: RSV, rhinovirus come from nasopharyngeal area through eustachian tube
61
Give 3 symptoms of acute otitis media.
1. Severe pain lasting weeks 2. Coryzal symptoms 3. Generally unwell. 4. Otorrhoea.
62
Otitis media investigations?
1. ALWAYS test function of facial nerve on examination 2. Otoscopy 3. Discharge sent for microscopy and culture
63
Describe the treatment for acute otitis media.
Watch and wait - most spontaneously resolve in 24 hrs Analgesia. If recurrent, offer antibiotics and consider a grommet.
64
Give 2 potential complications of acute otitis media.
1. Extra-cranial: mastoiditis, TM perforation. 2. Intra-cranial: meningitis, abscess.
65
What is the function of a grommet?
A grommet keeps the middle ear aerated and prevents the accumulation of fluid in the middle ear.
66
When might a grommet be indicated?
1. Recurrent AOM. 2. Chronic otitis media + effusion (glue ear) 3. ET dysfunction.
67
What is the common name for otitis media + effusion?
Glue ear.
68
What causes glue ear?
Infection! 45% follow AOM.
69
Presentation of glue ear? Examination findings?
Difficulty hearing and pressure sensation Examination: TM dull and light reflex is lost
70
Pathophysiology of glue ear?
inflammatory fluid build up leading to conductive hearing impairment via chronic inflammatory changes and eustachian tube dysfunction
71
Give 3 risk factors for glue ear.
* Bottle fed * paternal smoking * atopy * genetic disorders egCF and Downs
72
Causes of deafness in children? (infections)
* Temporary=OME * Meningitis, mumps and measles
73
Aetiology of tonsillitis
Viral mostly Bacterial-group A strep most common (S.pyogenes) Most common in age 5-15
74
Tonsillitis- bacterial vs viral assessment
Centor score: Age, exudate, tender/swollen anterior cervical LNs, temp over 38, absent cough
75
Management of tonsillitis
Viral is self limiting Bacterial- should swab for culture and commence antibiotics (usually benzylpencillin)
76
What are the criteria for considering a tonsillectomy?
1. \>7 episodes of acute tonsilitis in a year. 2. OSA or sleep-deprived breathing.
77
What is the epidemiology of periorbital cellulitis?
Peak in under 10s Males twice as commonly affected Peak occurrence in later winter and early spring
78
What are the causes of periorbital cellulitis?
S. pneumoniae and S. aureus
79
How to manage periorbital cellulitis?
If mild preseptal can do from home with broad spectrum empirical abx If more extensive- hospital, IV abx, supportive therapy If large abscesses, intracranial complications at px and in frontal sinusitis, need urgent drainage
80
Complications of periorbital cellulitis?
* Visual related: (vision loss is 11%) start to lose red colour vision (sign of optic nerve compromise) * Neurological complications: sepsis, intracranial abscess, cavernous sinus thrombosis
81
Describe the types of manifest and latent strabismus
Manifest: Esotropia=eye turns in Exotropia=eye turns out Hypertropia=eye goes up Hypotropia=eye goes down Latent: Esophoria=inwards under occluder Exophoria=outwards under occluder Hyperphoria=up under occluder Hypophoria= down under occluder
82
what tests to identify strabismus
``` Cover test (manifest squint) Cover/uncover test (latent squint) ```
83
What shape lens for myopia? For hypermetropia?
Myopia/short sighted= concave Hypermetropia/long sighted=convex
84
Causes of microcytic anaemia?
* Iron deficiency * thalassaemia * chronic inflamm
85
Two microcytic anaemias and how you could tell them apart?
Iron-deficiency anaemia (high TIBC on iron profile) Thalasaemia (low TIBC on iron profile)
86
What is the most common cause of anaemia in children?
Iron deficiency.
87
Why are kids more susceptible to iron deficiency anaemia than adults?
30% of their iron comes from diet and 70% from recycled rbcs (whereas adults have 5% from diet and 95% from recycling) Have higher expenditure
88
Give 3 signs of anaemia in children.
1. Pallor. 2. Irritable. 3. Lethargy. 4. SOB. 5. Tachycardic 6. Murmur 7. Poor growth
89
Risk factors for iron-deficiency anaemia in infants and children?
Infants = maternal iron deficiency, premature, low birth weight, multiple pregnancy, exclusively breastfed after 6 months Children=veggie/vegan, GI disorders, chronic blood loss
90
What is the treatment for iron deficiency anaemia? Side effect?
* Diet advice, ferrous sulphate tablets * Constipation
91
Why does the treatment for iron deficiency anaemia sometimes fail?
Non-compliance.
92
Give 3 signs of beta thalassaemia major.
1. Severe anaemia. 2. Jaundice. 3. Splenomegaly. 4. Failure to thrive. 5. Present at 6months+ (when adult Hb takes over)
93
Describe the management of beta thalassaemia major.
- Genetic counselling. - Blood transfusions with iron chelation to prevent overload
94
Long term sequelae of untreated thalassaemia major?
Progressive severe anaemia Try to compensate with bone marrow expansion and extramedullary haematopoiesis
95
What may carriers of thalassaemia have?
Mild microcytic hypochromic anaemia
96
Which type of thalassaemia is generally incompatible with life?
alpha major (4/4 genes missing to make alpha chain)
97
What does a low reticulocyte count indicate?
A production problem e.g. non-haemolytic. Infection Chronic inflamm Bone marrow malignancy Bone marrow failure (aplastic)
98
What does a high reticulocyte count indicate?
A degradation problem e.g. bleeding or haemolysis.
99
What are some types of haemolytic anaemia?
Intrinsic to RBC: * sickle cell * enzyme deficiencies: G6PD, pyruvate kinase * spherocytosis Extrinsic: * auto and alloimmune * DIC * TTP * malaria
100
How do haemolytic anaemias present?
* Splenomegaly * Cholecystitis/gall stones * Jaundice (hyperbilirubinaemia) * Increased lactate dehydrogenase * Leg ulcers
101
Describe the molecular structure of haemoglobin.
Four protein chains (globin) each surrounding a non-protein group (heme).
102
What is the difference between adult and fetal haemoglobin?
Adult Hb: the four protein chains are made up 2 alpha and 2 beta chains. Feta Hb: the four protein chains are made up of 2 alpha and 2 gamma chains.
103
Describe the inheritance pattern seen in sickle cell disease.
Autosomal Recessive.
104
When do symptoms of sickle cell disease start to present? WHy?
Between 3 and 6 months as HbF levels fall
105
Pathophysiology of sickle cell disease?
Sickling causes deformed and easily destroyed rbcs= occlusion of microcirculation and chronic haemolytic anaemia
106
Give 3 consequences of sickle cell disease.
1. Life expectancy 20 years younger 2. Anaemia. 3. Infection. 4. Painful crises. 5. Stroke. 6. Acute chest infection/infarction. 7. Aplastic crises 8. Splenic sequestration.
107
What is the affect of sickle cell disease on Hb and reticulocyte count?
Low Hb. Raised reticulocyte count.
108
Describe the management for sickle cell disease.
- Hydroxycarbamide. - Transfusions. - Stem cell transplants. -prophylactic penicillin + pneumococcal, influenza and meningococcal vaccines to prevent infections
109
How does hydroxycarbamide help in sickle cell disease?
Increases foetal haemoglobin production
110
What is the advantage of being a sickle-cell carrier?
Protected against malaria.
111
Why are children with sickle cell more predisposed to infection? How is this prevented?
Microvascular occlusion leads to splenic infarction leads to increased susceptibility to infection, specifically bacterial sepsis. Prevented by: -prophylactic penicillin -pneumococcal, influenza and meningococcal vaccines.
112
What are some triggers for a painful crisis in sickle cell?
cold dehydration infection hypoxia
113
What is acute chest syndrome in sickle cell?
Occurs after painful crises. Presents with pain, resp distress, hypoxia, chest X ray signs.
114
How can you spot aplastic crises in a child with sickle cell?
- very anaemic - low reticulocyte count (normally high in sickle cell)
115
Cause of aplastic crises in sickle cell?
Parvovirus B19 (slapped cheek)
116
Most common hereditary/intrinsic haemolytic anaemia in Europeans?
Spherocytosis.
117
How can spherocytosis be treated?
Splenectomy - can increase RBC survival.
118
Describe the inheritance pattern seen in hereditary spherocytosis.
Autosomal Dominant.
119
Describe the inheritance pattern for G6PD?
X-linked recessive
120
What can trigger haemolysis in G6PD?
* eating broad beans * infection * antimalarials
121
What is haemolytic disease of the newborn?
transplacental maternal antibodies causing alloimmune haemolysis of foetal rbcs Most commonly due to rhesus alloimmunisation (Rh + rbcs from foetus enter rh - maternal blood circulation)
122
Why does haemolytic disease of newborn occur then and not in utero?
In utero, bilirubin cleared by placenta In neonate, liver does clearing and can't handle high bilirubin load
123
Presentation of haemolytic disease of the newborn?
Jaundice Pallor Hepatosplenomegaly Severe= oedema, ascites, petechiae
124
What anaemia will haemolytic disease of newborn present as?
Normocytic. Increased reticulocyte count
125
Management of haemolytic disease of newborn?
``` 50%= normal Hb and bilirubin and need monitoring for late onset anaemia at 6-8 weeks 25%= mod disease and may require transfusion 25%= severe disease= stillborn or have hydrops fetalis- require immediate resus, temp stabilisation, exchange transfusion. ```
126
What is fanconi anaemia?
X-linked/autosomal recessive condition Bone marrow failure, solid tumours, leukaemia
127
How to manage fanconi anaemia?
Treat specific symptoms in each patient Only curative= stem cell transplant Cancer treatment Surgery for skeletal malformations
128
What are the liver dependent clotting factors?
10, 9, 7, 2 = "1972"
129
Name 2 coagulopathies.
1. Haemophilia. 2. Von Willebrand disease
130
what factor is deficient in haemophilia a
clotting factor 8 (classic haemophilia, most common)
131
Describe the inheritance pattern seen in haemophilia.
X linked recessive.
132
what factor is deficient in haemophilia b
clotting factor 9 (also known as christmas disease)
133
how does severe haemophilia present?
* Easy bruising * Mouth bleeds * Haematomas * Joint bleeds * Increased APTT
134
how does mild/mod haemophilia present?
Delayed presentation until following trauma or bleeds with surgery/dental extractions
135
Management of haemohpilia?
Factor VIII for haemophilia a Factor IX for haemophilia b
136
How does von willebrand factor work?
Assists in platelet plug formation by attracting circulating platelets and binds to CF VIII, preventing its clearance from the plasma
137
Presentation of Von Willebrand disease?
Bleeding tendency from mucosa eg epistaxis/menorrhagia, spontaneous bleeding
138
How to manage von willebrand disease?
tranexamic acid and desmopressin or concetrates with vwf or factor VIII-vwf Testing for 1st degree relatives
139
When does immune thrombocytopenia occur?
Commonly after viral infection or sometimes after immunisation
140
What diagnosis would you suspect in a child with a single figure platelet count but is otherwise well?
ITP. They would have a normal blood film and clotting, just very low platelets.
141
Management of ITP?
* Most children will not need treatment and recover spontaneously in weeks-months. * Avoid NSAIDs and aspirin
142
Give 3 signs of thrombocytopenia.
1. Petechial rash. 2. Bruising. 3. Bleeding.
143
Give 2 causes of thrombocytopenia in children.
1. ITP. 2. Marrow failure.
144
What can trigger acute ITP?
Viral infection.
145
How to manage fanconi anaemia?
Treat specific symptoms in each patient Only curative= stem cell transplant Cancer treatment Surgery for skeletal malformations
146
Describe rickets presentation
Rachitic rosary, limb deformity, weakness, misery Metaphyseal swellings, bowing deformities, slowing of linear growth, motor delay, hypotonia, fractures, resp distress
147
Causes of low vit d/rickets?
Maternal vitamin D deficiency causes low stores in newborn, exclusive breastfeeding will exacerbate Lack of dietary intake ie prolonged unsupplemented breastfeeding
148
What is rickets?
Severe vitamin D deficiency (child version of osteomalacia)
149
Pathophysiology of rickets?
lack of vit D; inadequate mineralisation of bone matrix Decreased vit D \> decreased calcium and phosphate \> secondary hyperparathyroidism In children, this occurs before the growth plates have closed.
150
Presentation of rickets?
Hypocalcaemic seizures or tetany, bony deformity (eg genu varum and valgum), irritable and reluctant to weight bear, severe can result in cardiomyopathy Delayed walking, waddling gait, impaired growth, fractures, dental deformities
151
Investigating rickets?
Bloods, wrist X ray (long bone showing cupping, splaying and fraying of metaphysis eg champagne glass wrist) required for diagnosis
152
Management of rickets?
Diet, sunlight, vit D supplementation=oral calciferol and calcium supplement Maintenance dose of calciferol is recommended for family members
153
What are the 3 main differentials for a limping child?
1. Infection e.g. sepsis/osteomyelitis. 2. Trauma e.g. NAI, fracture. 3. Tumour.
154
What is the likely cause of a limp in a child aged 0-3?
* Trauma - toddlers fracture, NAI * Infection - osteomyelitis, **septic arthritis** * Displasia - DDH
155
What is the likely cause of a limp in a child aged 3-10?
* Trauma * Infection - **transient synovitis,** osteomyelitis * Perthe's disease.
156
What is the likely cause of a limp in a child aged 10-15?
* Trauma. * Infection - osteomyelitis * **SUFE** * Perthe's disease
157
What must you remember to consider as a differential in a limping child?
Intra-abdominal pathology e.g. hernia, testicular torsion.
158
What investigations might you want to do on a child presenting with a limp?
1. General observations e.g. HR, BP, T, RR, O2 sats. 2. FBC, BM, ESR and CRP. 3. XR - AP and lateral views of the the joint and the joints above and below. 4. USS - effusion in joints? 5. CT/MRI.
159
Give 3 signs of septic arthritis.
* Children under 2 * Systemically very unwell * Pain at rest * Raised WCC and CRP * Hip = flexed, abducted, externally rotated
160
Most common cause of septic arthritis?
S. aureus
161
Septic arthritis investigations and management?
FBC, ESR/CRP, synovial fluid examination and culture, blood cultures. Xray will show later changes (14-21 days) Surgical emergency: surgical drainage and IV antibiotics- cefuroxime
162
Describe Kocher's criteria. What is it used for?
For differentiating transient synovitis from septic arthritis. * non-weight bearing * temp\> 38.5 * ESR\>40 mm/hr * WCC\> 12000 cells/mm **3/4 = septic joint.**
163
What is DDH?
DDH - developmental dysplasia of the hip. Abnormal relationship of the femoral head to the acetabulum -\> aberrant development of the hip.
164
What tests can be done on clinical examination in the neonatal period to pick up DDH?
1. Ortolani test. 2. Barlow manoeuvre. Can be confirmed with USS.
165
Give 3 risk factors for DDH.
1. Female (M:F - 1:8). 2. First born. 3. Breech birth. 4. Family history.
166
age cut off for uss or pelvic x ray for DDH?
Under 4.5 months = USS Over 4.5 months= pelvic XR
167
Describe the management of DDH.
1. Pavlik harness. 2. Surgical reduction. Prevention = safe swaddling
168
What are the 2 main risks associated with the surgical management of DDH.
1. Avascular necrosis. 2. Re-dislocation.
169
What is the most common cause of hip pain/limp in children aged 3-10?
Transient synovitis
170
What is transient synovitis?
Acute onset joint inflammation following illness, often respiratory.
171
How does transient synovitis differ from septic arthritis?
Transient synovitis: * no pain at rest * systemically well * rest, physiotherapy and NSAIDs often help * no findings on esr/crp, xray, uss
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Transient synovitis management?
* no long term sequelae and self limiting * Simple analgesia, rest and physio * Usually resolves within 2 weeks
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What is Perthe's disease?
A self-limiting idiopathic disease characterised by avascular necrosis of the femoral head.
174
Key points in history for Perthe's disease?
* 5-10 yr olds * Developed over weeks * No history of trauma * All hip movements limited * May have referred pain to groin/thigh/knee * Systemically well
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Describe the management of Perthe's disease.
* If bone age under 6 years: activity restriction, physio, nsaids * If bone age over 6 years: surgery
176
Give 3 risk factors for Perthe's disease.
1. ADHD. 2. Deprivation. 3. Passive smoking. 4. LBW. 5. Short stature.
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What is SUFE?
Slipped upper femoral epiphysis - slippage of the femoral head
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Who is likely to be affected by SUFE?
A pre-pubescent obese male.
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How does SUFE present?
* several week history of vague groin/thigh discomfort * Drehmann's sign (passively flex hip, falls back into **external rotation** and abduction)
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What is the treatment for SUFE?
Surgical pinning of the hip.
181
Give 3 signs of osteomyelitis in children.
1. Joint pain. 2. Lethargy. 3. Fever.
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Name an organism that commonly causes osteomyelitis in children.
Staphylococcus aureus.
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Most common site of osteomyelitis in children?
Distal femur and proximal tibia
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Pathophysiology of osteomyelitis?
Infection of bone marrow, commonly S. aureus Inflammatory destruction of bone. If periosteum involved: necrosis and detachment forming a sequestrum Bony remodelling causes deformity NB/ involucrum= viable periosteum separated from underlying bone and forms new bone around it
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What is the likely mechanism of osteomyelitis in children?
Haematogenous spread- tends to occur in rapidly growing and highly vascular metaphysis of growing bones (long bones more common)
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Investigating osteomyelitis?
FBC, ESR/CRP, blood cultures, bone cultures (gold standard), MRI
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Management of osteomyelitis?
Local bone and soft tissue debridement, stabilisation of bone, local antibiotic therapy, reconstruction of soft tissue, reconstruction of osseous defect zone Antibiotic therapy 4-6 weeks if acute, at least 12 weeks if chronic IV cefuroxime or IV flucloxacillin, switch to PO if improving.
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What is Kohler's disease?
Osteochondrosis of tarsal navicular bone A non inflammatory, none infectious derangement of bony growth, affecting the epiphyses
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Presentation of kohler's disease?
Unilateral antalgic gait, local tenderness of medial aspect of foot over navicular bone
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Management of kohler's disease?
Rest, analgesia, avoid excessive weight bearing, short leg cast for immobilisation, treat for at least 6 weeks Chronic course but rarely over 2 years
191
3 types of discoid meniscus?
Incomplete (bit thicker and wider than normal), complete (tibia completely covered by meniscus) and hypermobile wrisberg (normal shape but no posterior attachment to tibia)
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How will a discoid meniscus present?
More prone to injury than normal shaped meniscus Some may never experience problems Most cases= knee problems, vague pain, audible snap on terminal extension, swelling, locking
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Management of discoid meniscus?
If asx, do nothing otherwise, arthroscopic partial meniscectomy and rehab
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What is osgood schlatter disease?
Self limiting disorder of the knee generally in active adolescents before tibial tuberosity has finished ossification, during adolescent growth spurt
195
Presentation of osgood schlatter disease?
Gradual onset pain and swelling below knee, relieved by rest, provoked by knee extension or hyperflexing while prone
196
Management of osgood schlatter disease?
Conservative: rest, ice, physio and exercise advice, simple analgesia Most patients able to return to activity after 2-3 weeks
197
What is the criteria for making a clinical diagnosis of juvenile idiopathic arthritis?
Joint swelling/stiffness \>6 weeks in children \<16 and no other cause is identified.
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What symptoms are associated with JIA?
1. Fever. 2. Salmon-pink rash. 3. Uveitis. 4. Pain. 5. Morning stiffness. 6. Swelling.
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Give 5 potential consequences that can occur if you fail to treat JIA.
1. Damage. 2. Deformity. 3. Disability. 4. Pain. 5. Bony overgrowth. 6. Uveitis.
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Subtypes of juvenile idiopathic arthritis?
Oligoarticular (1-4 joints) Polyarticular (at least 5 joints)- RF positive or negative Systemic Psoriatic Enthesitis-related Undifferentiated
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Qualifying factors for it to be systemic JIA?
at least 2 weeks of fever with at least one of: rash, ln enlargement, hepato/splenomegaly, serositis= pericarditis/pleuritis/peritonitis
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Qualifying factors for it to be psoriatic arthritis (JIA)?
Arthritis and psoriasis or arthritis and at least 2 of: dactylitis, nail pitting, onycholysis, psoriasis in first degree relative
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Qualifying factors for it to be enthesitis related arthritis?
Arthritis or enthesitis and 2 of: sacroiliac/lumbosacral pain, HLA b27 positive, family history HLA b27 related disease, anterior uveitis
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Describe the non-medical treatment for JIA.
* Education, support, liaison with school * Physiotherapy
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Describe the medical treatment for JIA.
1. Steroid joint injections. 2. NSAIDS. 3. Methotrexate. 4. Systemic steroids. 5.biologics- etanacerpt.
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What extra-articular features might you see in someone with JIA?
1. Psoriasis. 2. Dactylitis. 3. Nail pitting. 4. Rash. 5. Fluctuating fever. 6. Uveitis.
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Name a severe, potentially life-threatening complication of systemic onset JIA.
Macrophage-activation syndrome (MAS).
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What signs might you see in someone with macrophage-activation syndrome (MAS)?
- High fever. - Hepatosplenomegaly. - CNS dysfunction. - Purpuric rash. - Cytopaenia.
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What is the treatment for macrophage-activation syndrome (MAS)?
- Supportive treatment. - Steroids.
210
Give 5 differentials for a painful joint.
Life-threatening differentials: - Leukaemia. - Septic arthritis. - NAI. Pain and swelling: - Trauma. - Infection. - Reactive arthritis. - JIA. Pain and no swelling: - Hypermobile joint syndrome. - Perthe's disease.
211
Define child development.
The biological, psychological and emotional changes that occur between birth and adolescence as the individual progresses from dependency to increasing autonomy. It is a continuous process with a predictable sequence however each child's development is unique.
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Give 5 influences on a child's development.
1. Genetic factors. 2. Stimulating environment. 3. Pregnancy factors e.g. premature? Mums health? 4. Healthy attachment. 5. Medical conditions. 6. Abuse/neglect/domestic violence. 7. Healthy peer relationships. 8. Education. 9. Nutrition. 10. Parenting style.
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What are the 4 domains of child development?
1. Gross motor. 2. Fine motor and vision. 3. Speech, language and hearing. 4. Social interaction and self care skills.
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What are the developmental milestones for gross motor function?
* Newborn: flexed arms and legs, equal movement in all 4 limbs * 3m: lifts head on tummy. * 6m: chest up with arm support, can sit unsupported. * 9m: craws, pulls to stand * **12m: walking** * 2 years: walks up stairs, runs * 3 years: jumps
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With regards to gross motor development, at what age would you expect a child to do the following: a) walking. b) jumping. c) crawling. d) walking up stairs.
a) Walking - 12 months. b) Jumping - 3 years. c) Crawling - 8 months. d) Walking up stairs - 2 years.
216
What are the developmental milestones for fine motor and visual function?
* 6 weeks: fix and follow * 6 mo: palmar grasp (picks up cube) * 1 yr: pincer grip * 1.5 yrs mo: tower of 3 cubes * 2 yrs: tower of 6 cubes, draw straight line * 3 yrs: bridge of 3 cubes, draw circle
217
With regards to fine motor and visual development, at what age would you expect a child to do the following: a) drawing with crayons. b) building a tower of 8 cubes. c) takes an object in each hand. d) builds a tower of 2 cubes.
a) Drawing with crayons - 12m. b) Building a tower of 6 cubes - 2 years. c) Takes an object in each hand - 6m. d) Builds a tower of 3 cubes - 18m.
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What are the developmental milestones for speech, language and hearing?
* newborn: startles to loud noises * 6 mo: babbles, turn to sound * 9 mo: responds to own name, 'dada', 'mama' * **12 mo: 1 proper word** * 18 mo: **nouns** e.g. body parts, teddy * 2 years: **verbs** e.g. eat, 50+ words, simple sentences (give teddy) * 3 years: **adjectvies** e.g. colours, speech mainly understandable * 4 years: can count 5 objects
219
With regards to speech, language and hearing, at what age would you expect a child to do the following: a) form short sentences and name body parts. b) knows colours and can count. c) laughs and squeals. d) has mainly understandable speech.
a) Forms short sentences and name body parts - 2 years. b) Knows colours and can count - 4 years. c) Laughs and squeals - 3 months. d) Has mainly understandable speech - 3 years.
220
What are the developmental milestones for social interaction and self-care skills?
6 weeks: smile spontaneously 6 mo: finger feeds 9/10 mo: wave bye bye, stranger danger **12 mo: drink from cup, uses spoon/fork** 2 years: undresses, dry by day 3 years: parallel play, interactive play evolving, put on t shirt 4 years: play simple board game, gets dressed themself
221
With regards to social interaction and self-care skills, at what age would you expect a child to do the following: a) uses cutlery. b) plays with others, names a friend. c) smiles. d) waves bye-bye.
a) Uses cutlery - 12 months. b) Plays with others, names a friend - 3 years. c) Smiles - 6 weeks. d) Waves bye-bye - 9/10 months.
222
What does 'The healthy child programme' encourage?
1. Encourages care to keep children healthy and safe. 2. Promotes healthy eating and activity. 3. Identifies problems in children's development. 4. Identifies 'at risk' families for more support. 5. Ensures children are prepared for school.
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Gross motor developmental red flags?
1. Not sitting by 12 months. 2. Not walking by 18 months.
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Fine motor developmental red flag?
Hand preference before 18 months (cerebral palsy)
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Speech and language developmental red flag?
No clear words by 18 months - ASD? Language problems?
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3 social developmental red flags?
* No smiling by 3 months * No response to carers interactions by 8 weeks. * No interest in playing by 3 years.
227
Give 5 causes of developmental delay.
1. Genetics. 2. Pregnancy. 3. Factors around birth. 4. Factors in childhood. 5. Environmental.
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Causes of developmental delay: give examples of genetic causes.
1. Chromosomal disorders e.g. Down's syndrome. 2. Single gene disorders e.g. Duchenne. 3. Polygenic e.g. ASD, ADHD. 4. Micro-deletions or micro-duplications.
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Causes of developmental delay: give examples of pregnancy related causes.
1. Congenital infections e.g. CMV, HIV. 2. Exposure to drugs/alcohol e.g. FAS. 3. MCA infarct.
230
Causes of developmental delay: give examples of birth related causes.
1. Prematurity. 2. Birth asphyxia (due to hypoxia).
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Causes of developmental delay: give examples of medical causes that may occur during childhood.
1. Infections e.g. meningitis. 2. Chronic illness. 3. Hearing or visual impairment. 4. Acquired brain injury.
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How might you investigate developmental delay?
Thorough history and examination. Tailor any investigations to the child e.g. - Boys not walking by 18m check creatinine kinase for Duchenne. - Focal neurological signs -\> MRI brain. - Genetic testing. - Unwell, failure to thrive -\> metabolic investigations. There is no 'developmental screen', investigations need to be tailored towards to the child.
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Causes of developmental delay: give examples of environmental causes.
1. Abuse and neglect. 2. Low stimulation.
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What is global developmental delay?
Significant delays in at least two of the four areas of development.
235
Types of epilepsy in childhood?
Absence seizures Childhood epilepsy syndrome ('benign' if can predict from EEG that will stop by certain age) Infantile spasms=West syndrome Bects: Rolandic epilepsy (benign epilepsy with centro-temporal spikes) Juvenile myoclonic epilepsy
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Define seizure.
A paroxysmal abnormality of motor, sensory, autonomic and/or cognitive function due to transient brain dysfunction. -may be epileptic or non-epileptic
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Types of seizures? (epileptic and non-epileptic)
_Epileptic_ * Epilepsies * Acute asymptomatic seizures * Febrile seizures _Non epileptic_ * convulsive syncope e.g. cardiac syncope, neurally-mediated, hypovolaemic, expiratory-apnoea syncope (blue breath holding spells)
238
Define convulsion.
A convulsion is a seizure, either epileptic or non-epileptic, with motor components e.g. * stiffening=tonic * a massive jerk=myoclonic * jerking=clonic * thrashing about=hypermotor as opposed to a non-convulsive seizure with motor arrest e.g. unresponsive stare, drop attack
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Define epileptic seizure.
What makes a seizure eplieptic is the nature of the underlying **electival activity** in the brain: **excessive, hypersynchronous** neuronal discharges in all or part of the cerebral cortex.
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Define epilepsy.
An umbrella term brain disorders that predispose the patient to having epileptic seizures.
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How long do epileptic seizures tend to last for?
30 - 120 seconds.
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Give 3 signs of epileptic seizures.
1. Movement. 2. Tongue biting. 3. Head turning. 4. Muscle pain.
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Causes of epilepsies?
**-genetic** (70%): caused by allelles at several lociso inhertiace is complex **-structural, metabolic:** cerebral dysgenesis, damage (vascular occlusion, infection)
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What are febrile convulsions?
Febrile convulsions are epileptic seizures accompanied by fever. They usually occur early in viral infection and tend to be brief generalised tonic-clonic seizures.
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Px of febrile convulsion?
Last less than 5 mins, child becomes stiff, then limbs twitch, lose consciousness and may wet/soil themselves May be sick, foam at mouth and eyes may roll back Sleepy for up to an hour after event
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What is a complex febrile seizure?
Lasting longer than 15 mins
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How long do non-epileptic seizures tend to last for?
1 - 20 minutes.
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Give 3 signs of non-epileptic seizures.
1. Eyes closed. 2. Talking/crying. 3. Pelvic thrusting.
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What 2 broad categories can epileptic seizures be divided into?
Generalised and focal.
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Give 3 examples of generalised seizures.
1. Absence. 2. Myoclonic. 3. Tonic. 4. Atonic. 5. Generalised tonic-clonic.
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What are absence seizures?
Seizures where there is a transient loss of consciousness with an abrupt onset and termination. Momentary unresponsive stare with motor arrest, lasts \<30s. Developmentally normal but can interfere with school.
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Give some possible side effects of the treatment for absence seizures in children?
* Sodium Valporate - weight gain, hair loss, teratogenic. * Ethosuxamide N+V
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How might you investigate suspected absence seizures?
. Observe an episode - hyperventilation, ask the child to blow on a windmill. 2. EEG - would show 3-second spike and wave discharges.
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Absence seizure meds?
1. Sodium valproate/ ethusoxemide for girls 2. Clobazam
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What would you expect to see in a tonic seizure?
Generalised increase in tone.
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What can absence seizures evolve into?
Juvenile myoclonic epilepsy (JME).
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What are the signs of juvenile myoclonic epilepsy?
Clumsiness and GTCS that occur shortly after waking and are often provoked by sleep deprivation.
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What would you expect to see in a generalised tonic-clonic seizure?
Sudden onset rigid phase followed by a convulsion in which the muscles jerk rhythmically.
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Treatment for tonic-clonic seizures in children?
1. Sodium valproate, carbamazepine for girls 2. Clobazam
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* Side effects of first line treatment for tonic-clonic seizures? * Contraindication?
* Sodium valproate - weight gain, hair loss, teratogenic * Carbmazepine - rash, hyponatraemia, can interfere with contraception. Contraindicated in absence and myoclonic seizures.
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What would you expect to see in a myoclonic seizure?
Isolated muscle jerking.
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First and second line AEDs for focal seizures?
1. Lamotrigine/carbmazepine, sodium valproate (not for girls) 2. Clobazam
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What investigations might you want to do in someone presenting with seizures.
1. Eye witness account/video is invaluable! 2. ECG. 3. EEG. 4. MRI or CT.
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Why must you do an ECG in those suffering from seizures?
To check for arrhythmia as the cause e.g. long-QT syndrome.
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Give 3 potential side effects of AED's.
1. Cognitive disturbances 2. Heart disease. 3. Drug interactions. 4. Teratogenic.
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Name common epilepsy syndromes in order of increasing age.
* 3-12 months: Infantile spasms (west syndrome) * 1-3 yrs: Lennox-Gaustat * 4-10 years: Benign rolandic epilepsy (15%), childhood absence epilepsy (2%) * 10-20 yrs: Juvenile absence, juvenile myoclonic -name does not necesarily correspond to seizures e.g. can have absence and tonic-clonics in "juvenile myoclonic epilepsy"
267
Name 3 conditions hat are commonly misdiagnosed as epilepsy.
1. Sandifer syndrome. 2. Benign neonatal sleep myoclonus. 3. Syncope.
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What is syncope?
Insufficient blood or O2 supply to the brain causes paroxysmal changes in behaviour, sensation and cognitive processes.
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What non-neurological disease is sandifer syndrome associated with?
GORD. Patients present with GORD and a characteristic neck movement disorder.
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Common causes of ‘funny turns’? (paroxysmal disorders)
* blue breath holding spells (toddlers) * reflex anoxic syncope (triggered by pain, fright, discomfort) * syncope * migraine * vertigo
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Describe a floppy infant.
Hypotonia, weakness, ligamentous laxity, increased range of joint mobility
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What features suggest central hypotonia/UMN in children?
Normal strength, dysmorphic features, normal/brisk tendon reflexes, irritability +/- loud cry, history suggestive of HIE (hypoxic-ischaemic encephalopathy)/birth trauma/sxs hypoglycaemia,seizures Central=2/3 cases, commonly HIE
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What are indicators of peripheral hypotonia/LMN in children?
Decreased strength, reduced/absent reflexes, fasciculation, myopathic face, weak cry
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Causes of floppy infant?
Central hypotonia=acute encephalopathies (HIE, hypoglycaemia, intracranial haemorrhage), chronic encephalopathies (cerebral malformations, metabolism errors, chromosomal disorders, endocrine disorders, metabolic disorders), connective tissue disorders (Ehler-Dahlos, OI) Peripheral hypotonia= spinal cord (syringomyelia), anterior horn cell (spinal muscular atrophy), NMJ (MG), muscular disorders (dystrophies, myopathies), peripheral nerves, metabolic myopathies
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What is cerebral palsy?
Non progressive cerebral pathology that leads to a disorder of movement and posture in children. Damage to immature brain (most between 24 weeks and term)
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Why might the clinical signs of cerebral palsy change over time?
The clinical signs may change over time as the brain matures but the underlying aetiology is not progressive.
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Classification of cerebral palsy?
* Spastic * athetoid (hyperkinesia) * ataxic * mixed
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Give 3 causes of cerebral palsy.
80% antenatal - hypoxia, infection, haemorrhage, ischaemia. 10% peri-natal - hypoxia, infection, haemorrhage. 10% postnatal - hypoxia, infection e.g. meningitis, haemorrhage, encephalopathy, trauma.
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Presentation of cerebral palsy?
Motor features- mono/hemi/para/quadriplegia Don't meet developmental milestones eg not sitting by 8 months, not walking by 18 months, early hand preference before 1 year GORD, epilepsy, vomiting, constipation, bladder issues, drooling, orthopaedic problems all common
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How to diagnose cerebral palsy?
Definitive dx may not come until 12-18 months Clinical observation and parental observation
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Describe the support that is offered to someone with cerebral palsy.
1. Physiotherapists for mobility and hand function. 2. SALT for communication. 3. Feeding support. 4. Sleeping support. Support for children with disabilities needs to be holistic, child focused and with an MDT approach.
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What drug can be used to treat hypertonia in children with cerebral palsy?
Botox
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Fits, faints and funny turns causes?
In sleep: benign neonatal sleep myoclonus and parasomnias On feeding: GORD and sandifer syndrome Fever: febrile seziures, vaso-vagal syncope Pain/shock/startle: reflex asystolic syncope, cyanotic breath holding, hyperekplexia Tired/bored/stress: self gratification behaviour, tics, daydreaming Excitement: shuddering spells, cataplexy
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Potential childhood motor disease?
Tourette syndrome and tics, tremor, dystonia, ataxia, restless legs syndrome, myoclonus, juvenile huntington disease
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DSM-V criteria for ADHD
Symptoms must be present before age 12, and present in at least 2 settings, affect functioning. At least 6 symptoms of inattention for at least 6 months or At least 6 symptoms of hyperactivity for at least 6 months
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Non drug management of ADHD
parent education and training CBT can be offered Meds not advised for pre-school children Advice: plan day, clear boundaries, brief and specific instructions, incentive schemes, kep social situations short and sweet, exercise, healthy diet, bedtime routine, help at school
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Medical management of ADHD
Stimulant drugs (increase dopamine): Short acting methylphenidate eg ritalin Long acting methylphenidate eg delmosart Lisdexamfetamine Non stimulant drugs (reduced breakdown of noradrenaline): Atomoxetine (SNRI), guanfacine
288
Describe autism spectrum disorders
Abnormal development from under 3 years old in: * social interaction * communication * restricted, stereotyped and repetitive behaviour Diagnosis requires at least 6 symptoms across 3 core areas
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Management of autism spectrum disorders
Non-pharm management behavioural therapies, social skills groups, OT, communication interventions, input from dietician if needed, specialised educational programs and structured support
290
ICD-10 for anorexia nervosa
``` Deliberately keeping weight below 85% of expected via restricted diet choice, excessive exercise, induced vomiting, use of appetite suppressants and diuretics Dread of fatness-intrusive overvalued idea Endocrine effects (menstruation stops/puberty delayed if menarche not yet achieved, loss of sexual interest in men) ```
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How to screen for eating disorders?
SCOFF Do you make yourself sick because you're uncomfortably full? Do you worry that you've lost control over how much you eat? Have you recently lost more than one stone (about 6kg) in 3 months? Do you believe you are fat when others say you are thin? Would you say that food dominates your life?
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Potential clinical signs of anorexia nervosa?
Dry skin, lanugo hair, orange skin and palms, cold hands and feet, bradycardia, drop in BP on standing, oedema, weak proximal muscles (squat test)
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Management of anorexia nervosa?
* *1. Family therapy.** 2. IPT. 3. CBT. Weight restoration at 0.5kg/week - monitor for re-feeding syndrome.
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Define bulimia nervosa
Binges followed by compensatory weight loss behaviours eg self induced vomiting, fasting, intensive exercise, abuse of medication BMI maintained at over 17.5
295
Describe the aetiology of ADHD.
- Genetic and environmental. - Neuroanatomical and neurochemical factors too. - CNS insults e.g. FAS or premature.
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ASD signs: communcation, social interaction and social imagination
* Communication: non-verbal communication challenging, only communicate needs, no understanding of jokes * Interaction: poor eye contact, struggles to understand social roles * Imagination: no imaginative play, obsessions/rituals struggles with change
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RF's for developing anorexia?
1. Social pressure. 2. Perfectionist traits. 3. Family attitudes to food. 4. Low self esteem. 5. Occupation/interests. 6. Family history.
298
Give 5 symptoms of depression.
1. Loss of interest. 2. Fatigue. 3. Poor sleep. 4. Reduced appetite. 5. Low concentration. 6. Feelings of guilt and self blame. 7. Low confidence. 8. Agitated. 9. Hopeless.
299
Describe the non-medical and medical treatment of depression.
Non-medical: Education, CBT, IPT and family therapy. Medical: fluoxetine, sertraline, citalopram.
300
Give 3 predisposing factors for a child developing depression.
1. Family history. 2. Stress in pregnancy. 3. Poor attachment. 4. Poverty. 5. Isolation.
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Give 3 precipitating factors for a child developing depression.
1. Trauma. 2. Drugs. 3. Infections. 4. Puberty. 5. Exam stress. 6. Sexual abuse. 7. Bullying.
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Give 3 perpetuating factors for a child developing depression.
1. Chronic illness. 2. Malnutrition. 3. Ongoing neglect. 4. Ongoing poverty.
303
Describe the foetal circulation.
Placenta -\> umbilical vein -\> IVC -\> RV -\> foramen ovale -\> LA -\> aorta -\> umbilical arteries -\> placenta. OR: ... RV -\> pulmonary artery -\> ductus arteriosus -\> aorta ...
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What is the function of the foramen ovale and the ductus arteriosus in the foetal circulation?
They are used to bypass the non-functiong lungs.
305
Name 4 congenital heart problems that can cause a L -\> R shunt.
1. VSD. 2. ASD. 3. AVSD. 4. PDA.
306
Most common septal defect?
VSD
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Give 5 signs of a VSD.
1. Poor feeding and FTT. 2. Tachypnoea. 3. Thrill. 4. Pan-systolic murmur. 5. Increased work of breathing.
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You request a CXR for a patient with a VSD. What would you expect to see?
1. Cardiomegaly. 2. Pulmonary oedema. 3. Enlarged pulmonary arteries.
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Gold standard diagnosis of septal defect?
Echo
310
Murmur for VSD and where to listen?
* VSD = very systolic = pansystolic. * Best heard over tricuspid area (lower left sternal border)
311
Management of VSD?
Qp/Qs (pulmonary to systemic blood flow ratio) of at least 2.0 requires surgical repair Aim to close large VSDs within first 2 years of life 75% of small VSDs close spontaneously by age 10
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Why are ASD's often asymptomatic?
ASD's are often asymptomatic because the blood flow in the atria is low pressure and so breathlessness etc is uncommon.
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Most common ASD?
Patent foramen ovale.
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Type of murmur in ASD?
* a split double s2 * best heard in pulmonary area (upper left sternal edge)
315
RFs for ASD
* maternal smoking in first trimester * maternal diabetes * maternal rubella * maternal drug use eg cocaine/alcohol
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Give 5 signs of a PDA.
1. Poor feeding, FTT. 2. Tachypnoea. 3. Active precordium. 4. Thrill. 5. Continuous machinery murmur.
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Management of ASD?
If less than 5mm, spontaneous closure within 12 months Surgical closure required if bigger than 1cm
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Describe the management for congenital health defects that cause a L-\>R shunt.
1. Stabilise the patient. 2. Increase calorie intake. 3. NG tube. 4. Diuretics and ACEi to prevent HF symptoms. 5. Surgical repair.
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AVSD is a common defect in people with which chromosomal abnormality?
Trisomy 21 (Down's syndrome).
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Describe the pathaphysiology of an AVSD? 2 types
Failure of endocardial cushions to fuse properly Complete AVSD: large left to right shunt causes excessive pulmonary blood flow leading to HF and pulmonary vascular resistance Partial AVSD: left to right shunt and volume overload of RA and RV but the pulmonary artery pressure is only mildly elevated
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Presentation of AVSD?
Tachypnoea, tachycardic, poor feeding, sweating, failure to thrive, all with a complete AVSD are symptomatic by 1 year Prominent sternal heave, murmur
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Murmur in AVSD and where to auscultate?
Ejection systolic murmur in pulmonary area (left upper sternal border) Mid-diastolic murmur over tricuspid area (left lower sternal border) Holosystolic murmur might be heard at apex
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Management of AVSD?
Surgical management as patients would die by age 2-3 without
324
Name 2 congenital heart problems that can cause a R -\> L shunt.
1. Tetralogy of fallot. 2. Transposition of the great arteries.
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What 4 components make up Tetralogy of fallot?
1. Ventricular septal defect 2. Pulmonary stenosis 3. Right ventricular hypertrophy 4. Overriding aorta Most common cyanotic congenital heart disease
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Give 3 signs of Tetralogy of fallot.
1. Cyanosis. 2. Collapse. 3. Acidosis.
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If tetralogy of fallot isn't managed, what can occur?
polycythaemia, cerebral abscess, stroke, infective endocarditis, HF, death
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Murmur in tetralogy of fallot and where to listen?
Pansystolic murmur best heard over mid or upper left sternal edge Smaller the VSD=louder the murmur
329
What are tet spells?
Hypoxic spells in tetralogy of fallot with peak incidence between 2-4 months of life: Paroxysm of hyperpnoea, irritability, increasing cyanosis
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Management of tetralogy of fallot?
Medical= squatting, prostaglandin infusion, beta blockers, morphine, saline bolus Surgical management for definitive repair or palliative management
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Which defects have a higher risk of HF?
Significant left to right shunt= VSD, ASD, PDA Transposition of the great arteries
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Describe the two types of transposition of the great arteries?
dextro-transposition=aorta is anterior and to the right of the pulmonary artery levo-TGA= aorta anterior and to the left of the pulmonary artery
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What is coarctation of the aorta?
Arterial duct tissue encircles the aorta at the point of insertion of the duct. When the duct closes, the aorta constricts, this causes severe obstruction to LV outflow.
334
When do you get cyanotic heart disease?
Absent/not wide enough tricuspid/pulmonary/aortic valve Coarctation of aorta Ebstein anomaly Tetralogy of fallot TGA Truncus arteriosus Anywhere with a right to left shunt
335
What is rheumatic fever?
Systemic illness 2-4 weeks after pharyngitis due to cross reactivity to group A beta-haemolytic streptococcus (S. pyogenes)
336
What are the diagnostic criteria for rheumatic fever? Describe
Revised Jones diagnostic criteria= positive throat culture or anti-streptolysin O/anti-DNA B titre and 2 major or 1 major+2 minor criteria
337
What are the major criteria in the revised jones criteria?
SPECS Sydenham's chorea, polyarthritis, erythema marginatum, carditis, subcut nodules
338
What are the minor criteria in the revised jones criteria?
CAPE CRP/ESR raised Arthralgia Pyrexia ECG-prolonged PR
339
Management of acute rheumatic fever?
Antibiotics=benzathine benzylpenicillin Aspirin/NSAIDs Assess for emergency valve replacement Secondary prevention with abx every 3-4 weeks
340
What factors lead to subacute bacterial endocariditis?
Endothelial damage, platelet adhesion, microbial adherence
341
What organisms cause IE?
S. aureus, S. viridans, S. pneumoniae, HACEK organisms
342
Presentation of IE?
persistent low grade fever, heart murmur, splenomegaly, petechiae/oslers nodes/janeway lesions/splinter haemorrhages
343
Criteria for diagnosis of IE? describe
Modified Dukes criteria 2 major 1 major and 3 minor 5 minor
344
Major criteria in modified dukes?
positive blood cultures, evidence of endocardial involvement (echo)
345
Minor criteria in modified dukes?
predisposition, fever, vascular phenomena, immunologic phenomena, microbiology evidence, other echo findings
346
Management of IE?
IV antibiotics May require surgical intervention
347
What is coarctation of the aorta?
Arterial duct tissue encircles the aorta at the point of insertion of the duct. When the duct closes, the aorta constricts, this causes severe obstruction to LV outflow.
348
Give 3 signs of coarctation of the aorta.
1. Radio-femoral delay. 2. Weak femoral pulses. 3. Difference in pre and post-ductal saturations.
349
Give 3 signs of aortic stenosis.
1. Palpable thrill. 2. Ejection systolic murmur. 3. LVH.
350
What is a possible consequence of persistent pulmonary hypertension, like that seen in CHD associated with a L-\>R shunt?
Eisenmenger syndrome: high pressure pulm. blood flow damages pulmonary vasculature -\> increased resistance (pulm. hypertension) -\> RV pressure increase -\> shunt direction reverses -\> CYANOSIS!
351
What is a possible consequence of persistent pulmonary hypertension, like that seen in CHD associated with a L-\>R shunt?
Eisenmenger syndrome: high pressure pulm. blood flow damages pulmonary vasculature -\> increased resistance (pulm. hypertension) -\> RV pressure increase -\> shunt direction reverses -\> CYANOSIS!
352
What factors aid reflux in GORD?
Low tone of muscular portion of lower oesophagus, short and narrow oesophagus, delayed gastric emptying, shorter lower oesophageal sphincter, liquid diet and high calorie requirement (distends stomach), significant periods recumbent
353
Presentation of GORD?
Distressed, unexplained feeding difficulties, hoarseness +/- chronic cough, single episode pneumonia, faltering growth, if able may report pain
354
When does GORD or regurgitation (=posseting) present normally
Within 2 weeks of life
355
How to manage a breast fed baby with gord
Use alginate eg gaviscon mixed with water after feeds
356
How to manage a formula fed baby with gord
``` 1= do not over feed (less than 150ml/kg/day) 2= decreased feed volume by increasing frequency (2-3 hourly) 3= use feed thickener 4= stop thickener and use alginate added to formula ```
357
What to use after alginate if no success in GORD?
histamine antagonists or ppi
358
Name 5 potential causes of poor feeding
Infection, metabolic disorders, genetic disorders, structural abnormalities, neurological disorders, mum and feeding technique
359
Presentation of pyloric stenosis
Baby starts bringing up small amounts of milk after feeding, which gets worse over a few days Then projectile yellow vomit (milk curdles in stomach acid) Reduced faeces If untreated: dehydration and not gaining weight
360
When does pyloric stenosis present
About 6 weeks after birth
361
You do a blood gas on a neonate with pyloric stenosis. What would it show?
Metabolic Alkalosis - low K+ and Cl-. The baby has vomited up all the HCl and the kidneys go into overdrive - increased K+ secretion.
362
How to manage pyloric stenosis
- IV fluids. - Repeat gases to monitor alkalosis. - Stop feeding to stop vomiting. - Pyloromyotomy once stable
363
You suspect pyloric stenosis in a neonate. What investigations might you do?
1. U+E. 2. Blood gas. 3. USS - hypertrophy of pyloric sphincter
364
Potential presentation of IBS
Bloating, cramping, chronic or intermittent diarrhoea/constipation, urgency with defecation, incomplete sensation of defecation, passage of mucus in stool May have dizziness, nausea, loss of appetite
365
How to manage IBS
Diet and lifestyle management eg small meals, more often, low fat and high carbs
366
How common is gastroenteritis?
Very! At least one episode per year for most children
367
Common causes of gastroenteritis
Rotavirus is most common infantile (NB rotarix oral vaccine at 8 and 12 weeks) Norovirus commonest acorss all age groups Campylobacter is most common bacterial cause Also E.coli and adenovirus
368
Presentation of gastroenteritis
Sudden onset diarrhoea +/- vomiting, abdo pain, mild fever (bloody diarrhoea if campylobacter- generally due to undercooked meat and unpasteurised milk)
369
Management of gastroenteritis
Self limiting Send a stool sample if ?septicaemia/blood or mucus in stool/immunocompromised Encourage fluids (not carbonated drinks) IV or oral therapy if dehydrated Give full strength milk asap Leave it 48 hours before returning to school
370
How long do sxs of gastroenteritis last?
Diarrhoea around 5-7 days Vomiting around 1-2 days
371
Potential complications of gastroenteritis?
Haemolytic uraemic syndrome, reactive complications eg Reiter's syndrome, toxic megacolon, acquired/secondary lactose intolerance
372
What counts as constipation in children?
Less than 3 complete stools/week Hard/large stool Rabbit droppings Overflow soiling in children over 1
373
Management of simple constipation in children?
Diet and lifestyle advice- high fibre, increase fluids Then add Macrogol eg Movicol Then add stimulant laxative eg Senna Consider adding in lactulose In secondary care can do manual evacuation, antegrade colonic enema, polyethylene glycol solutions
374
Describe the pathophysiology of appendicitis
``` Luminal obstruction (faecolith or lymphoid hyperplasia, impacted stool) Decreased venous drainage and localised inflammation by commensal bacteria causes and increased pressure. This causes ischaemia which can lead to necrosis and perforation ```
375
Presentation of appendicitis
Dull peri-umbilical pain transfers to a sharp right iliac fossa pain May have vomiting, anorexia, nausea, diarrhoea or constipation MAGNET: migration of pain to RIF, anorexia, guarding, nausea, elevated temp, tenderness in RIF
376
What can be found on examination of appendicitis
``` Rebound tenderness and percussion pain over McBurneys point and guarding Psoas sign (pain when patient lies on left side while right thigh is flexed backward) Rovsing's sign (pain in RLQ upon palpation of LLQ) ```
377
Management of acute appendicitis
Antibiotics and laparoscopic appendicectomy
378
Why are children at higher risk of appendix perforation?
Tend to have delayed presentation
379
Two types of childhood hernia?
Umbilical and epigastric
380
How to manage hernias in children?
Umbilical- surgery if still present after 3 years Epigastric- surgery only if uncomfortable or a nuisance
381
Describe how Crohn's disease affects the body (microbiology and location)
Remitting and relapsing disease, affects anywhere between the mouth and the anus Transmural inflammation, deep ulcers and fissures with cobblestone mucosa, skip lesions Non-caseating granulomatous inflammation
382
Presentation of Crohns disease
Abdominal pain and bloody/mucus containing diarrhoea Oral aphthous ulcers, perianal disease (tags, fistulae) Extra-intestinal features=MSK, eyes, skin, renal, hepatobiliary
383
Investigations for IBD?
Faecal calprotectin, routine bloods, stool microscopy and culture, colonoscopy with biopsy (=definitive diagnosis and can distinguish between UC and CD)
384
How to manage Crohn's disease? Acute attack
In acute attack: avoid anti-motility drugs eg loperamide as it can cause toxic megacolon In acute attack: fluid resus, nutritional support, prophylactic heparin, corticosteroid and immunosuppressant eg mesalazine or azathioprine
385
How to manage Crohn's disease? Maintenance
Azathioprine, smoking cessation Surgery can be used- ileocaecal resection, stricturoplasty, small/large bowel resections
386
Course and pathophysiology of ulcerative colitis?
Relapsing/remitting Diffuse continual mucosal inflammation of large bowel only. Begins in the rectum and spreads proximally. Mucosal and submucosal inflammation only, crypt abscesses and goblet cell hypoplasia
387
What is a protective factor against UC?
Smoking!
388
Presentation of UC?
Bloody diarrhoea, PR bleeding and mucus discharge, increased faecal frequency, urgency and tenesmus Extra-intestinal: MSK, skin, eyes, hepatobiliary
389
How to induce remission in UC?
Corticosteroid and immunosuppressant eg mesalazine or azathioprine
390
Maintenance management of UC?
Immunomodulators- mesalazine or sulfasalazine Next line=infliximab Surgery= ileostomy and complete resection= curative, but many have sub-total colectomy to begin with
391
Pathophysiology of Coeliac disease
T cell mediated autoimmune response. Response to gliadin and genetic (HLA-DQ2/DQ8) Anti-gluten CD4 t cell response, with anti-gluten antibodies, anti-tissue transglutaminase, endomysin antibodies and intraepithelial lymphocytes activated Causes epithelial cell destruction and villous atrophy
392
which HLA molecules is Coeliac Disease often associated with?
DQ2 and DQ8.
393
What are the forms of coeliac disease?
Classical, atypical, latent, silent, potential
394
What are potential extra-intestinal features of coeliac disease?
Dermatitis herpetiformis, osteoporosis, anemia, short, delayed puberty, arthritis
395
How to investigate for coeliac disease?
Serology Must have had gluten in diet for at least 6 weeks prior to testing IgA and IgAtTG, IgAEMA Duodenal biopsy
396
Common causes of failure to thrive
Down syndrome, cerebral palsy, heart disease, infections, milk allergy, CF, coeliac disease, GORD
397
What is marasmus
Protein and energy malnutrition. Loss of fat mass especially buttocks Chronic diarrhoea and emaciated body type There is NO oedema
398
What is marasmus usually a sign of?
Poor socioeconomic status, child abuse+neglect, lack of food resources
399
How to manage marasmus?
Similarly to kwashiorkor and reintroduce food slowly and correct any electrolyte imbalances
400
What is kwashiorkor
Inadequate protein with a reasonable calorie intake
401
Presentation of kwashiorkor
Fatigue, irritability, lethargy, failure to thrive, loss of muscle mass, generalised oedema, pot belly, fatty liver, prone to infections, hair and skin changes (erythematous, exfoliation, pigment, dry hair, thin nails)
402
What is seen in bloods for kwashiorkor?
low glucose, low protein, high cortisol and GH, low salt, iron deficiency anaemia, metabolic acidosis
403
Management of kwashiorkor?
Correct fluids/electrolytes- needs to be done in about 48 hours Reintroduce foods slowly afterwards using RUTF (ready to use therapeutic foods eg peanut butter, milk powder, sugar, vegetable oil, minerals and vitamins) Treatment takes around 2-6 weeks
404
Pathophysiology of Hirschprung's disease
``` Congenital aganglionic megacolon disease Aganglionic segment (short/long/total) remains in tonic state so there is a failure in peristalsis Functional obstruction due to accumulated faeces ```
405
Presentation of Hirschprung's disease
Abdominal distension, bilious vomiting, failure to pass meconium within 48 hours after birth Males 4 times more likely
406
What is a big complication of Hirschprung's disease
Hirschprung's enterocolitis: stasis allows bacterial proliferation, which can cause sepsis and death
407
How to investigate for Hirschprungs
Initially a plain X ray Gold standard=rectal suction biopsy
408
Management of Hirschprungs
IV antibiotics, NG tube, bowel decompression initially Definitive= surgery to resect aganglionic section and connect unaffected bowel to dentate line
409
What is intussusception? Who does it happen to?
* Movement/telescoping of one part of bowel into another \> obstruction, inflamm, bloody stools * Peak at 5-7 months, rare after 2 years, males twice as likely
410
Presentation of intussusception
1. Colicky pain. 2. Vomiting. 3. Abdominal mass. 4. Redcurrant jelly stool.
411
How to diagnose intussusception?
abdominal uss
412
How to manage intussusception?
1. Gas in anal canal to reduce intussusception. 2. Analgesia e.g. morphine. 3. IV fluids if shocked.
413
What is meckel diverticulum?
Outpouching of lower part of small intestine- a remnant of umbilical cord
414
Presentation of meckel diverticulum?
Most asx 1. Severe rectal bleeding. 2. Intussusception. 3. Volvulus.
415
What is malrotation with volvulus?
Occurs during embryonic development at about the 10th week Bowel twists so blood supply is cut off
416
Presentation of malrotation with volvulus?
* Obstruction with bilious vomiting * Bouts of crying * pull legs into body * not passing faeces
417
Give a potential consequence of malrotation.
SMA blood supply to the small intestine can be compromised -\> infarction.
418
How to manage malrotation with volvulus
Dx by Xray Tx= operation- stop necrosis of bowel and may need bowel resection
419
What is toddler diarrhoea
Chronic nonspecific diarrhoea, at least 3 loose watery stools/day Some alternate with constipation Self limiting at about age 5-6 NOT due to malabsorption or serious bowel problem
420
What are the 4Fs for diet consideration in toddler diarrhoea
Fat, fluid, fruit juices and fibre
421
What is colic
Self-limiting repeated episodes of excessive and inconsolable crying for at least 3hrs a day, at least 3 days a week for at least 1 week Occurs in up to 4 months of age
422
Presentation of colic
Often crying in late afternoon or evening, draws knees up and arches back
423
Management of colic?
REASSURANCE Soothing strategies, parental wellbeing aids Discourage simeticone (infracol) or lactase drops, probiotics, herbal supplements and maternal diet modification Exclude other possible causes of distress
424
Describe the three types of biliary atresia
Type I= common bile duct atresia with patent proximal ducts Type II= common hepatic duct atresia with cystic structures in porta hepatis Type III= R and L hepatic duct atresia to level of porta hepatis (most common)
425
What is biliary atresia?
Extrahepatic bile ducts obliterated by inflammation and subsequent fibrosis
426
Presentation of biliary atresia?
Presentation shortly after birth Persistent jaundice Pale stools and dark urine in term infants with normal birth weights
427
What investigations are required in biliary atresia and what results shown?
LFTs: increased GGT, TGs normal USS Liver histology from percutaneous biopsy
428
Management of biliary atresia?
Surgery, liver transplant needed before 8 weeks Prognosis without treatment is 18 months
429
Complications of biliary atresia?
Ascending cholangitis, cirrhosis, portal hypertension, liver failure, hepatocellular carcinoma
430
Pathophysiology of cow's milk protein allergy
Immune-mediated allergic response to casein and whey IgE mediated type 1 hypersensitivity or non-IgE(T cell activation)
431
Presentation of cow's milk protein allergy
Acute and rapid onset (less than 2 hrs after ingestion) * pruritus * erythema * Urticaria and lip swelling * acute colicky abdo pain * vomiting * diarrhoea * may have resp signs
432
Management of cow's milk protein allergy
Specialised formula feeds for at least 6 months/ 9-12 months old, then reevaluate
433
A 6-month old breast fed infant developed widespread urticaria immediately after the first formula feed. a) What investigation might you do? b) What is the most likely diagnosis?
a) Skin-prick test for cow's milk. b) IgE mediated cow's milk allergy.
434
A 4-month-old infant, formula fed since birth, has loose stools and faltering growth. Skin prick test to cow's milk is negative but elimination of cow's milk results in resolution of symptoms which return on trial re-introduction. What is the most likely diagnosis?
Non-IgE mediated cow's milk allergy.
435
Choledochal cysts pathophysiology?
Usually in extrahepatic ducts (CBD and HDs), sometimes in intrahepatic ducts Type I cysts are most common-lower end of CBD joins up with pancreatic duct: pancreatic juice and bile mix leading to a weakening of bile duct wall and ballooning- can lead to pancreatitis and cholangitis
436
Management of choledochal cyst
USS to identify Commonly incidental finding Surgery for removal of cyst
437
Presentation of choledochal cyst
May have jaundice, intermittent abdo pain, cholangitis, peritonitis, lump, pancreatitis
438
Aetiology of neonatal hepatitis syndrome
in 20%- viral (cytomegalovirus, rubella, hep a/b/c) 80%- nonspecific virus
439
Presentation of neonatal hepatitis syndrome
Usually at 1-2 months Jaundice, not gaining wt/ht, hepatosplenomegaly
440
Management of neonatal hepatitis syndrome
Ix: liver biopsy and bloods Mx: none specific, may need vitamins, potentially a liver transplant
441
Presentation of liver failure in children
Increased AST and ALT with prolonged clotting +/- jaundice +/- encephalopathy
442
Causes of liver failure
Viruses eg HSV, EBV, CMV, Hep A/B/E Inherited metabolic disorders eg Wilsons, mitochondrial Toxins Medication eg erythromycin Autoimmune Low blood flow eg HF Chronic: hep C, autoimmune, haemochromatitis, alpha 1 antitrypsin deficiency, CF, biliary atresia, sclerosing cholangitis
443
Give 3 signs of UTI in children.
1. Fever. 2. Miserable. 3. Vomiting. 4. Dysuria.
444
What investigations might you do on a child who you suspect has a UTI?
1. Urine dip. 2. MCS on clean catch urine. A sample can also be obtained using an in-out catheter. 3. USS KUB. 4. DMSA - renal scarring. 5. MCUG - reflux.
445
What is the treatment for UTI in children?
IV cefuroxime. Switch to PO trimethoprim if stable.
446
How would you treat a UTI that has been caused by ESBL e.coli?
You would give meropenem. ESBL bacteria are resistant to all penicillins and cephalosporins.
447
Presentation of pyelonephritis
At least 38 degrees and bacteriuria or Less than 38 degrees and loin pain/tenderness and bacteriuria
448
When would you expect children to be dry overnight
Age 5
449
Factors contributing to nocturnal enuresis
Small bladder, inability to recognise full bladder, hormones, UTI, sleep apnoea, diabetes, chronic constipation, structural problem in urinary tract or nervous system
450
Management of nocturnal enuresis
\<5= reassurance and advice Non drug treatment: fluid intake, diet, toilet behaviour, rewards system then enuresis alarm if not responding (still more than 1-2 bed wets in a week) Drug= desmopressin if over 5 +/- enuresis alarm Imipramine hydrochloride is last line
451
Causes of acute renal failure
Hypoperfusion (blood loss, surgery, shock) Blockage of urinary tract Nephrotoxic drugs Haemolytic uraemic syndrome (e.coli usually) Glomerulonephritis
452
Symptoms of acute renal failure
Haemorrhage, fever, rash, bloody diarrhoea, vomiting, anuria or polyuria, pallor, oedema, eye inflammation, stomach mass
453
Treatment of acute renal failure?
Treat underlying cause U+Es Dietary changes Antihypertensives Iv fluids Diuretics
454
How long for it to be classed as chronic renal failure? Potential causes?
Over 3 months Causes: long term blockage, alport syndrome, nephrotic syndrome, PKD, cystinosis, chronic conditions eg diabetes, untreated acute kidney disease
455
What is alport syndrome
Inherited condition- X-linked most common, but can be autosomal recessive/dominant Deafness, renal damage and eye defects
456
Treatment of chronic renal failure
Diuretics Dialysis and transplant Diet changes (limit protein, potassium, phosphorus, sodium) Growth promoters/bisphosphonates/iron tablets
457
What defines nephrotic syndrome
Generalised oedema, heavy proteinuria (\>200mg/mmol) and hypoalbuminaemia (\<25g/L) (Flattened podocytes allow protein leakage) (Normal protein less than 20mg/mmol, normal albumin 35-45g/L)
458
Causes of nephrotic syndrome Which is most common?
Minimal change disease=most common Others= congenital nephrotic syndromes, focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis
459
Management of nephrotic syndrome
High dose steroids- most respond well but some will have relapse (=steroid-resistant nephrotic syndrome)- in which case may need maintenance low dose steroids or immunomodulatory drugs eg cyclophosphamide Low salt diet, prophylactic abx (as they leak Igs and steroids!), immunisations Prednisolone for first episode 60mg/m2 for 4-6 weeks then wean down
460
What is minimal change disease
Microscopically damage not evident. Idiopathic Develop nephrotic syndrome more quickly but much more responsive to treatment
461
What is congenital nephrotic syndrome What is needed in management
Genetic condition (parents need 1 faulty and 1 healthy gene each to pass on) Requires frequent albumin infusions to aid growth and development Typically leads to renal failure
462
Causes of nephritic syndrome
Post-streptococcal glomerulonephritis, henoch schnolein purpura, alport syndrome, sle/lupus nephritis
463
Describe post-strep glomerulonephritis
10-14 days after skin/throat infection with group A strep Haematuria, oedema, decreased urine output, htn, proteinuria, tired Normally self limiting so needs supportive tx only If strep infection persisting= penicillin
464
What is henoch schnolein purpura
IgA vasculitis Rash of raised red or purple spots usually on legs and bottom (extensor surfaces)
465
Px of henoch schonlein purpura
Rash, arthritis, abdo pain, kidney impairment (self limiting and mild usually), may get PR bleeding and intussusception Usually all preceded by 2-3 weeks of fever, headache, arthralgia/myalgia, abdo pain
466
Mx of Alport syndrome
ACEi/ARB, diuretics, limit sodium Monitor for potential renal failure
467
Mx of Lupus nephritis
Immunosuppressants eg cyclophosphamide or hydroxychloroquine, antihypertensives, may need steroids
468
Types of hypospadias When does it form
Subcoronal (near head of penis) Midshaft Penoscrotal (where penis and scrotum meet) Weeks 8-14 of pregnancy
469
Management of hypospadias
Surgery between 3 and 18 months, may need to be done in stages
470
Types of renal malformation?
Autosomal recessive polycystic kidney disease Duplication (single renal unit with more than one collecting system) Fusion (kidneys joined, but ureters enter bladder on each side)- most common= horseshoe Malrotation (little clinical significance) Multicystic dysplastic kidney (nonfunctioning renal unit but contralateral kidney normally fine) Renal agenesis (bilat=fatal, unilateral) Renal ectopia=kidney fails to ascend from true pelvis Renal hypoplasia (underdeveloped)
471
Pathophysiology of vescioureteral reflux
Urine flows from bladder back into ureters, potentially causing infection Shorter than normal attachment between ureter and bladder and incompetent valve
472
Describe the different grades of vesicoureteral reflux
I=into ureter II=into ureter and renal pelvis without distension III=into ureter and renal pelvis with mild swelling IV= mod swelling V=severe swelling and twisting or ureter
473
Management of vesicoureteral reflux
Can go away by itself (junction gets longer with age) Encourage frequent voiding May need surgery to stop reflux
474
Triad of haemolytic uraemic syndrome
Coombs test negative, thrombocytopenia, AKI Typically a/w e.coli
475
What is haemolytic uraemic syndrome
Toxins bind from e.coli causing thrombin and fibrin to be deposited in microvasculature RBCs damaged while passing through occluded small vessels causing haemolysis
476
Px haemolytic uraemic syndrome
Profuse diarrhoea turning bloody 1-3 days later, fever, abdo pain and vomiting
477
Mx of HUS
Supportive- fluid and electrolyte mx, dialysis PRN, antihypertensive, keep circulating volume up
478
General management of eczema?
Avoid triggers, keep nails short, emollients Rest of treatment depends on severity: may include corticosteroids, abx, antihistamines, calcineurin inhibitors, phototherapy, immunomodulators or biologic medications
479
What steroid would you give for mild eczema?
Hydrocortisone 1%
480
What steroid for moderate eczema?
Betamethasone valerate 0.025% or clobetasone butyrate 0.05%
481
What steroid for severe eczema?
Betamethasone valerate 0.1% May need maintenance steroids or topical calcineurin inhibitors eg tacrolimus
482
What is Stevens-Johnson syndrome?
Toxic epidermal necrolysis. Rare, acute and potentially fatal skin reaction with sheet-like skin and mucosal loss Nearly always caused by medications
483
What medications commonly cause Stevens-Johnson syndrome
Cotrimoxazole, penicillin, cephalosporins, anti-convulsants, allopurinol, paracetamol, NSAIDs Usually within the first week of abx therapy
484
Presentation of Stevens-Johnson syndrome
Initially a flu-like illness Then, tender/painful red skin rash on trunk. extending to face and limbs over a course of hours-days (Skin lesions: macules/diffuse erythema/targetoid/blisters) The blisters merge to form sheets of skin detachment At least 2 mucosal surfaces affected eg conjunctivitis/mouth ulcers/cough/diarrhoea Acute phase lasts 8-12 days
485
Management of Stevens Johnson syndrome
Skin biopsy shows keratinocyte necrosis SCORTEN illness severity score to predict mortality Cessation of suspected causative drug, admission, analgesia, temp maintenance, nutrition and fluids General care of affected mucosal surfaces
486
Classification of allergic rhinitis?
Seasonal (if due to grass and tree pollens=hay fever) Perennial (throughout year, due to dust mites and animal dander) Intermittent (less than 4 days/week or less than 4 consecutive weeks) Persistent (more than 4 days/week or more than 4 consecutive weeks) Occupational
487
Management allergic rhinitis? Mild-mod
Nasal irrigation with saline Allergen avoidance PRN antihistamine- intranasal as first line eg azelastine Can use 2nd generation non sedating oral eg loratidine or cetirizine for mild-mod symptoms
488
What can be used in mod-severe allergic rhinitis?
Intranasal corticosteroid during periods of allergen exposure eg mometasone fluroate or fluticasone propionate
489
For hayfever, what pollens affect you when?
Tree=early-late spring Grass=late spring-early summer Weed=early spring-late autumn
490
What causes urticaria?
Histamine release Due to: allergens, bee and wasp stings, autoimmune/idiopathic (chronic), physical triggers eg cold/friction, medications
491
Types of antihistamine
Non sedating, sedating, short and long acting=H1 antihistamine H2 antihistamines
492
What is angio-oedema?
Swelling deep to the skin, commonly soft areas of skin eg eyelids and lips, more painful than weals/hives and takes longer to clear.
493
Presentation of anaphylaxis
Skin features-urticaria, erythema, flushing, angio-oedema Involves at least 1 of Resp/CV/GI system
494
How to manage anaphylaxis
ABCDE, high flow oxygen, IM adrenaline, repeat 3-5 mins if required, treat a drop in BP with fluid bolus, consider salbutamol if wheeze, antihistamines for itch(cetirizine) Prescribe an epipen
495
What are the most common vascular birth marks?
Macular stains or salmon patches: Angel's kisses (forehead/nose/upper lip/eyelids- disappear with age) or Stork Bites (back of neck, disappear with age) Hemangioma (grow rapidly 6-9 months then shrink and lose red colour) Port-wine stain/nervus flammeus (grows with the child, often face/arms/legs, flat pink/red/purple mark)
496
What are the most common pigmented birth marks?
Moles/congenital naevi, cafe au lait spots, mongolian spots (blue-grey spots on lower back or buttocks, common on darker skin)
497
Causes of infectious rash in children
Meningococcal, steven-johnsons syndrome, impetigo, kawasaki, staphylococcal scalded skin syndrome, eczema herpeticum, erythema nodosum, erythema multiforme, measles, glandular fever, hand foot and mouth disease, erythema infectiosum, chicken pox, nappy rash, scabies, tinea corporis, tinea capitis, mollusucm contagiosum
498
What are the three phases in Kawasaki disease?
Acute phase=weeks 1-2 Subacute phase= weeks 2-4 Convalescent phase=weeks 4-6
499
Pathophysiology of kawasaki disease?
Idiopathic. Not contagious. systemic vasculitis Mainly affects under 5s.
500
What are NICE criteria for diagnosis of kawasaki?
Over 38 degrees for at least 5 days and at least 4 key symptoms: Bilateral conjunctival infection, mouth/throat changes, hand and feet changes, rash, swollen glands
501
How to manage kawasaki disease?
IV immunoglobulin and high dose PO aspirin (to prevent thrombosis)
502
What should you be aware of when giving children aspirin?
Reyes syndrome: persistent vomiting, lethargy, liver and brain damage
503
Presentation of measles
Initially coryzal sxs, fever, small grey-white spots in mouth (Koplik spots) lasting a few days 2-4 days later= maculopapular rash on head/neck and spreads to rest of body
504
Management of measles
Self limiting viral illness and usually clears in about 7-10 days Mx: stay at home for at least 4 days from when rash first appears, antipyretic, fluids, gently clear crust from eyes, hot steam PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months
505
What are some complications of measles?
dehydration, otitis media, conjunctivitis, laryngitis, airway infections, febrile seizures
506
What causes chicken pox?
Varicella zoster virus
507
Presentation of chicken pox?
Fever, aches and headache for 1 day preceding rash: small itchy blisters that scab over
508
Management of chickenpox?
``` AVOID aspirin (reye's syndrome) Antipyretic, emollient, sedating antihistamine at night, keep nails short, dress comfortably, fluids Stay off school until all blisters crusted over ``` If an at risk group eg under 1 month, immunocompromised, severe heart/lung disease=aciclovir or vaccination
509
When are you infectious with chickenpox?
7-21 day incubation Infectious from 2 days before spots appear until all blisters crusted over
510
What type of infection is rubella? When is peak incidence?
Viral. Mainly in spring and early summer
511
Presentation of rubella?
Prodromal phase: low grade fever, headache, mild conjunctivitis, anorexia, rhinorrhoea Rash occurs 14-17 days after exposure: pink discrete macules that coalesce behind ears and on face, spreads to trunk then extremities Lymphadenopathy-cervical, suboccipital, post auricular
512
Management of rubella?
PCR is gold standard diagnosis Keep out of school for 4 days after rash appears, antipyretics, ask re contact with pregnant women PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months
513
What causes diphtheria?
Gram positive aerobic bacillus Corynebacterium diphtheriae causing acute upper resp tract infection
514
Presentation of diphtheria?
Early sxs=coryzal Nasal discharge- watery then purulent and bloody Membranous pharyngitis with fever, enlarged cervical LNs, oedema of soft tissues= bull neck appearance Cutaneous infection: pustules rupture causing punched out ulcers often on lower legs, feet and hands, with surrounding oedematous pink/purple skin. Takes 2-3 months to heal leaving a depressed scar May cause difficulty swallowing, paralysis and HF
515
Management of diphtheria?
PREVENTION: 6-in-1 vaccination at 2, 3 and 4 months Antibiotics (eyrtho/azithro/clarithro/penicillin), reinforcing vaccine dose, manage contacts with abx prophylaxis Booster vaccinations: first between 3.5 and 5 years, second between 13-18
516
Pathophysiology of scalded skin syndrome?
Staphylococcal disease. Also known as Ritter disease and Lyell disease Affects under 5s, especially neonates normally Exotoxins from S. aureus bind to desmosomes, causing skin cells to become unstuck- causes red blistering skin
517
Presentation of scalded skin syndrome?
Fever irritability and widespread erythema, then 24-48 hours later=fluid filled blisters form and rupture easily Nikolsky sign positive= gentle strokes causes exfoliation
518
Investigations for scalded skin syndrome?
Tzanck smear, skin biopsy, bacterial culture
519
Management of scalded skin syndrome?
Admit to hospital IV antibiotics eg flucloxacillin Supportive -antipyretic, fluids, petroleum jelly Healing usually complete within 5-7 days of starting treatment
520
Pathophysiology of whooping cough?
Bordetella pertussis bacterial infection Gram negative bacillus. Toxins paralyse cilia and promote inflammation
521
Describe the first phase of whooping cough?
1st phase=catarrhal phase for 1-2 weeks Coryzal sxs: rhinitis, conjunctivitis, irritability, sore throat, low grade fever, dry cough
522
Describe the second phase of whooping cough?
2nd phase=paroxysmal for 2-8 weeks Severe paroxysms of coughing followed by inspiratory gasp (whoop) NB/ in infants under 3 months the whoop is less common and apnoea is more common. Paroxysms more common at night and often followed by vomiting
523
Describe the third phase of whooping cough?
Convalescent phase=up to 3 months
524
Investigations for whooping cough?
If cough for less than 2 weeks: nasopharyngeal aspiratr If cough for more than 2 weeks: anti pertussis toxin IgG serology in under 5s or toxin detection in oral fluid for 5-17year olds FBC=lymphocytosis
525
Management of whooping cough?
Hospital if under 6 months and acutely unwell, sig breathing difficulties, feeding difficulties, complications eg pneumonia Macrolide antibiotic if cough for less than 21 days= clarithromycin (under 1 month), azithromycin otherwise Supportive NB/ abx don't alter course once disease established PREVENTION= vaccination at 2,3,4 months and booster at 3 years 4 months
526
Presentation of polio?
Fever, tiredness, headache, vomiting, neck stiffness and arm/leg pain In 1 in 200 cases, affects NS leading to temporary or permanent paralysis
527
Management of polio?
No treatment; need physio and OT PREVENTION= vaccination at 2,3,4 months, 3years 4 months and 13-18 years
528
How to diagnose TB in children?
Mantoux test= positive tuberculin skin test IGRA CXR History of contact Lab cultures very difficult to do in kids
529
TB drugs?
RIPE Rifampicin, isoniazid, pyrazinamide, ethambutol
530
Who gets BCG vaccination?
If born in areas of UK where rates are high If a parent/grandparent born in a country with high TB rates
531
Causes of meningitis in children?
Bacterial: Mainly N. meningitidis and S. pneumoniae In neonates= GBS (group B strep from mum's vagina) Viral: HSV, enterovirus and VZV
532
What is the presentation of meningitis in neonates and babies?
Non-specific! Eg Hypotonia, poor feeding, lethargy, hypothermia, bulging fontanelle
533
How to investigate for meningitis?
Kernigs and brudzinski's signs LP if under 1 month and fever, 1-3 months fever and unwell, under 1 year with unexplained fever and other features of serious illness
534
Management of meningitis?
Primary care: benzylpenicillin Secondary care bacterial: If under 3 months: cefotaxime and amoxicillin (to cover listeria) Over 3 months=ceftriaxone Dexamethasone QDS for 4/7 if LP suggestive bacterial meningitis PEP for contacts: ciprofloxacin Viral=aciclovir
535
What CSF findings are suggestive of bacterial meningitis?
Cloudy, high protein, low glucose, high neutrophils, cultured for bacteria
536
What CSF findings are suggestive of viral menigitis?
Clear, normal protein, normal glucose, lymphocytosis, negative culture
537
Causes of encephalitis?
Most common=HSV 1(children) and HSV 2(neonates- from genital warts) Others= VZV, CMV, EBV, enterovirus, adenovirus, influenza virus
538
Presentation of encephalitis?
Altered consciousness, altered cognition, unusual behaviour, acute onset focal seizures and focal neurological symptoms, fever
539
Management of suspected encephalitis?
Ix: LP (CSF and PCR) or CT if LP CI. MRI after LP, swabs, HIV testing Mx: aciclovir for HSV/VZV or ganciclovir (CMV) Repeat LP to ensure successful tx
540
How does HSV1 affect children?
Infectious for 7-12 days Commonly gingivostomatitis: fever, restless, dribbling, swollen gums which bleed easily, foul breath, ulcers on tongue/throat/palate/inside cheeks, enlarged LNs
541
Management of HSV?
Can use antivirals eg aciclovir/valaciclovir but for mild/uncomplicated eruptions require no treatment
542
What is slapped cheek syndrome caused by?
Also known as fifth disease/erythema infectiosum Parvovirus B19
543
When are the peak incidences for slapped cheek syndrome?
april and may
544
Presentation of slapped cheek syndrome?
Commonly 3-15 years old Rash=bright red scald-like on 1 or both cheeks, painless May have mild coryzal symptoms 1 in 4 will be asx By time rash develops, no longer infectious
545
Management of slapped cheek syndrome?
None needed. Caution around pregnant women
546
Types of impetigo and what caused by?
Non-bullous (about 70% cases)= S.aureus, S.pyogenes or both Bullous= S.aureus
547
Presentation of impetigo?
Non-bullous= golden/brown crusts from vesicles/pustules, commonly on face, limbs and flexures, may be mildly itchy Bullous-more common in infants= blisters rupture leaving flat yellow/brown crust, may have systemic features
548
Management of impetigo?
Good hygiene, stay away from school until healed or 48 hours after abx Localised non-bullous=hydrogen peroxide 1% cream (2nd line=fusidic acid 2%) Widespread non-bullous or bullous=oral flucloxacillin
549
How to manage candida?
topical antifungal eg miconazole gel If vaginal (v. uncommon before puberty)= fluconazole
550
Causes of toxic shock syndrome?
Staph or strep- from female barrier contraceptives, tampons, injury on skin, childbirth, nasal packing, ongoing staph or strep infection
551
Presentation of toxic shock syndrome?
Fever, flu-like sxs, N+V, diarrhoea, widespread sunburn like rash. lips/tongue/whites of eye turning bright red, dizziness and syncope, dyspnoea, confusion
552
Management of toxic shock syndrome?
Antibiotics and fluids
553
What causes scarlet fever?
Group A strep- affects people with recent strep throat or impetigo. Children over 3 at risk
554
Symptoms of scarlet fever?
Sudden fever a/w sore throat, lymphadenopathy, headache, N+V, loss of appetite, swollen and red strawberry tongue, abdo pain, malaise 12-48 hours after fever=rash: starts below ears/neck/chest/armpit/groin)- scarlet blotches (boiled lobster appearance). Then looks like sunburn with goose pimples, pastia lines (red streaks from ruptured capillaries)
555
Management of scarlet fever?
Penicillin Mostly follows benign course
556
What is Coxsackie's disease?
Hand foot and mouth disease, generally affects under 10s. Group A coxsackie virus- faecal-oral transmission
557
Presentation of coxsackie's disease?
Prodromal phase then tender oral ulcerative lesions and maculopapular lesions on hands and feet
558
Management of coxsackie's disease?
Supportive mx-fluids, soft diet, antipyretic analgesics, don't need to isolate the child Symptoms improve within 3-6 days with full resolution within 7-10 days
559
Red flags for primary immunodeficiency in kids?
faltering growth, need IV abx for infections, FH, at least 4 infections a year, at least 2 sinus infections a year, at least 2 pneumonias in past 3 years, frequent deep tissue abscesses, persistent fungal infections (over 6 months), at least 2 deep seated infections over 3 years, at least 2 months on at least 2 abx with little effect
560
Diagnosis of diabetes in children?
Random plasma glucose \> 11.1 mmol/L if symptomatic
561
Normal fasting blood glucose? post prandial glucose? ketones?
Fasting: 3.5-5.6mmol/L Post prandial: \<7.8 Ketones: \<0.6
562
DKA management?
Fluid, insulin, monitor glucose hourly, monitor electrolytes especially potassium and ketones 2-4 hourly, hourly neuro obs
563
Fluids in DKA?
0.9% NaCl 20ml/kg bolus if shocked, 10ml/kg if not shocked Maintenance and deficit fluids 5% fluid deficit in mild DKA 7% deficit in moderate 10% deficit in severe Do no subtract boluses given for shock
564
Initial management of type I diabetes mellitus in kids?
Aim for glucose 4-7: need to be able to check this Give insulin (pump or injection): basal bolus regime most common Carb counting Advice for hypos Lifestyle advice
565
Carb counting in DMT1?
Insulin:carb ratio= 1 unit for every 15 grams of carb Correction factor= 1 unit insulin brings blood glucose down by 8 Aim for glucose of 6
566
Presentation of hypoglycaemia?
Autonomic fxs: irritable, hungry, nauseous, shaky, anxious, sweaty Neuroglycopenic fxs: confused, drowsy, headache, coma, seizures
567
Management of hypoglycaemia?
Check levels to confirm Glucose tablets/gel/containing food or drink first Check glucose in 15 mins Follow up with longer acting carb (bread or biscuit) Severe hypo (requiring additional assistance from another person)= IM glucagon
568
Long term management of diabetes?
Aim for HbA1c less than 48mmol/mol Monitoring at least annually: eyes, urine, feet, BP, injection sites, bloods
569
Types of cryptorchidism?
True undescended= lies along normal path of descent but never present in scrotum Ectopic testis= lies outside normal path and outside of scrotum Ascending testis=previously in scrotum but higher now Absent/atrophic testis=non palpable testis was in scrotum at birth but later disappears
570
How to manage ?cryptorchidism?
6-8 week baby check exam If not descended, re examine at 4-5 months to see if spontaneous descent If not surgical treatment (orchodexy) before 12 months of age
571
Pathophysiology of testicular torsion?
Testis twists around spermatic cord and cuts off the blood supply to the testicle
572
Presentation of testicular torsion?
Sudden severe pain on one side of the scrotum, scrotal skin swells and turns red Nausea and vomiting potential May have just abdominal pain
573
Management of testicular torsion?
Surgical emergency: blood supply must be restored within 6 hours or the testicle will infarct If surgery delayed past 12 hours, 75% will need orchidectomy Newborns with torsion- testes almost always infarcted so need orchidectomy
574
What is a bell clapper deformity?
No tissue holding the testes to the scrotum so testes swing inside the scrotum= higher risk of torsion
575
Cut offs for precocious puberty?
Girls under 8 Boys under 9
576
2 types of precocious puberty?
Gonadotrophin dependent/ central precocious puberty= true precocious puberty with premature activation of the HPG axis Gonadotrophin independent/precocious pseudopuberty= due to increased production of sex hormones
577
Causes of central precocious puberty?
Mainly idiopathic or abnormalities of CNS
578
Causes of precocious pseudopuberty?
CAH, tumours (HCG secreting), McCune- Albright syndrome, silver-russell syndrome, testotoxicosis, exogenous androgen exposure, severe hypothyroidism
579
What can you give for true precocious puberty?
GnRH agonists
580
Define delayed puberty
``` Girls= absence of breast development by 13 or primary amenorrhoea with normal breast development by 15 Boys= absence of testicular development by age 14 ```
581
Causes of delayed puberty
Most common= constitutional delay in growth and puberty (CDGP) Central causes= CDGP, chronic illness, malnutrition, excessive exercise, psychosocial deprivation, steroids, hypothyroid, tumours, congential anomalies, irradiation treatment, trauma, idiopathic hypogonadotrophic hypogonadism Peripheral causes= bilat testicular damage, syndromes eg Prader-Willi, Klinefelter, rugs. irradation, Turner syndrome, intersex disorders, PCOS
582
How to manage delayed puberty?
Most are CDGP so need reassurance and monitoring Can start course of sex hormones (low dose testosterone or gradual increase of oestrogen then cyclical progestogen)
583
Causes of congenital hypothyroidism
Thyroid dysgenesis, thyroid hormone metabolism disorders, hypothalamic/pituitary dysfunction,
584
What happens in congenital hypothyroidism isn't treated?
cretinism: long term irreversible neurological problems and poor growth with intellectual disability spasticity, gait problems, dysarthria, profound mental disability
585
How to treat congenital hypothyroidism?
levothyroxine titrated to TFTs
586
Define gonadotrophin deficiency/hypogonadotrophinism
Low serum concentrations of LH and FSH in presence of low circulating concentrations of sex steroids
587
How to manage gonadotrophin deficiency?
Induce and maintain secondary sexual characteristics- testosterone and hCG or oestrogen and progestogen
588
What is Kallmann syndrome?
Hypothalamic gonadotrophin releasing hormone deficiency (GnRH) and deficient olfactory sense (hyposmia or anosmia) X linked recessive or autosomal recessive Greater penetrance in males
589
Presentation of Kallmann syndrome?
``` Females= primary amenorrhoea Males= cryptorchidism, micropenis ``` Most are normal stature Lots have associated stigmata- nerve deafness, colour blind, cleft palate, renal abnormalities
590
How to manage Kallmann syndrome?
Exogenous sex steroids to induce and maintain secondary sexual characteristics
591
What is congenital adrenal hyperplasia?
Group of autosomal recessive disorders of cortisol biosynthesis+/- aldosterone +androgen excess 21-hydroxylase deficiency
592
What is the classic form of CAH?
Ambiguous external genitalia in girls Boys have no signs at birth
593
What is the salt-losing form of CAH?
Boys present at 7-14 days with vomiting weight loss, lethargy, hyponatraemia, hyperkalaemia and dehydration Girls will be identified before a crisis due to genitalia
594
How to manage classic CAH?
Glucocorticoid replacement= hydrocortisone +/- Mineralcorticoids= fludrocortisone Salt losing form need NaCl Consider surgery for genitalia
595
Types of androgen insensitivity syndrome? describe
Complete=testosterone has no effect so genitals are entirely female Partial= testosterone has some effect so genitals not as expected eg penis underdeveloped, fully/partial cryptorchidism, some female genitalia.
596
How will females with complete androgen insensitivity syndrome present? and partial?
Complete=Develop breasts but amenorrhoea, no pubic or axillary hair, no womb or ovaries Partial=enlarged clitoris, undescended testicles, hypospadias, often raised as boys
597
Causes of childhood obesity?
Diet, exercise lack, sleep deprivation (low leptin and high ghrelin), genetics, socio-economics, medication, endocrine eg PCOS/hypothyroid/Cushings/Prader-Willi
598
Name 2 drugs that can be given as meningitis prophylaxis.
1. Rifampicin. 2. Ciprofloxacin.
599
2-years-old, 2m history of malaise, pallor and reduced appetite. Occasional febrile episodes associated with a pink rash and soreness in her left thigh. Now reluctant to weight bear despite walking at 13 months. All immunisations are up to date and developmental history shows no concerns. O/E: low grade fever and cervical lymphadenopathy. ESR is significantly raised. Give 3 differentials.
1. JIA. 2. ALL. 3. Transient synovitis. 4. Septic arthritis.
600
3-year-old, 7-day history of high fevers. Now developed red eyes, a rash, sore mouth and throat. Miserable and unwell with a diffuse maculopapular rash on his torso. He has bilateral injected conjunctiva, red cracked lips and a strawberry tongue. 3x2cm cervical swelling and swollen reddened palms. Give 3 differentials.
1. Kawasaki disease. 2. Scarlet fever. 3. Shingles/chickenpox. 4. Measles - ask about immunisation history.
601
Commonest cancer in children?
Leukaemia- ALL and then AML
602
Pathophysiology of acute lymphoblastic leukaemia?
Disruption of lymphoid precursor cells in the bone marrow leading to excessive production of immature blast cells: causes drop in functional RBCs/WBCs/platelets
603
Presentation of leukaemia in children?
Anaemia (lethargy, pallor) Thrombocytopenia (easy bruising/bleeding) Leukopenia (fevers, infections) May complain of bone pain Non specific sxs (weight loss, malaise)
604
Investigations for leukaemia?
FBC (pancytopenia, thrombocytopenia with significant lymphocytosis), serum biochemistry Blood films (blast cells) CXR (exclude mediastinal mass) Bone marrow aspirate confirms diagnosis LP
605
Management of leukaemia in children?
Chemo, supportive care with blood products, prophylactic anti-fungals Chemo in 5 phases (induction, consolidation, interim maintenance, delayed intensification, maintenance) Maintenance= 2 years for girls and 3 years for boys Stem cell transplants in high risk patients in 1st remission or relapsed patients
606
Name 5 different types of primary brain tumour?
Astrocytoma, medulloblastoma, diffuse midline glioma, ependymoma, craniopharyngioma, embryonal tumours, pineoblastoma, brainstem glioma, choroid plexus carcinoma, germ cell tumour
607
What is the most common brain and spinal cord tumour in children (apart from mets)?
Astrocytoma
608
Presentation of brain tumour?
morning headaches, nausea/vomiting, diplopia, seizure, increased head circumference, bulging fontanelle, signs of raised intracranial pressure
609
4 main types of astrocytoma?
Grade 1=pilocytic astrocytoma Grade 2=diffuse (fibrillary) astrocytoma Grade 3=anaplastic astrocytoma Grade 4=glioblastoma multiforme 80% are low grade (1 or 2)
610
What is the most common high grade brain tumour in children?
Medulloblastoma (2nd most common overall) Most commonly found in cerebellum
611
What is Wilm's tumour? How presents?
Most common paediatric renal mass= nephroblastoma Painless upper quadrant abdominal mass, haematuria in 20%, hypertension in 25%, ,may have acquired von willebrand disease
612
how to manage wilm's tumour?
Nephrectomy and chemo Cure in about 90% cases
613
How does neuroblastoma present?
Presents late- majority of symptoms due to mass effect or mets. Systemic fxs, bruising, weakness/paralysis/BB dysfunction, bone pain, abdominal distension, htn, horner's syndrome Almost all are stage 3 or 4 at diagnosis
614
Where does neuroblastoma arise from?
Adrenal or paraspinal sites most common arises from developing sympathetic nervous system
615
Investigating neuroblastoma?
FBC, esr, pt, catecholamine by products in urine, CT, MRI for paraspinal lesions, biopsy
616
Presentation of retinoblastoma?
Leukocoria (abnormal appearance of pupil), visual deterioration, red and irritated eye, faltering growth, squint, absent red reflex
617
Management of retinoblastoma?
Eye conserving treatments eg cryo/thermotherapy, photocoagulation, chemo Enucleation for all unilateral tumours filling over half of the eye or invading eye structures and subsequent fitting of prosthesis V sensitive to radiotherapy
618
Which childhood cancer has the best overall survival?
Retinoblastoma (about 99%)
619
What are the 2 main types of primary bone tumour in in children?
Osteosarcoma (more common) or ewing's sarcoma (v rare)
620
Presentation of hepatoblastoma?
Abdominal mass +/- poor appetite, weight loss, lethargy, fever, vomiting, jaundice
621
Investigating hepatoblastoma?
Raised alpha fetoprotein
622
What immunisations at 8 weeks?
6 in 1 rotavirus men B
623
What is contained in the 6 in 1 vaccination?
diphtheria, hepatitis b, Hib, polio, tetanus, whooping cough
624
What immunisations at 12 weeks?
6 in 1, PCV (pneumococcal), rotavirus
625
What immunisations at 16 weeks?
6 in 1, Men B
626
What immunisations at 1 year?
Hib/Men C, MMR, PCV, MenB
627
What immunisations at 3 years 4 months?
MMR, 4 in 1 pre school booster (diphtheria, tetanus, whooping cough, polio)
628
What immunisations at 12-13 years?
HPV
629
What immunisations at 14 years?
3 in 1 teenage booster (tetanus, diphtheria, polio), MenACWY
630
What is klinefelter's syndrome?
Extra X chromosome (commonly 47XXY)
631
How does klinefelter's syndrome present in kids?
Delayed speech or learning difficulties, rapid growth in mid-childhood or truncal obesity, hypogonadism Small firm testes, decreased facial and pubic hair, loss of libido, impotence, gynaecomastia, history undescened testes Infertility
632
Diagnosis of klinefelter's? Management?
Low/low-normal testosterone, elevated FSH and LH, chromosomal analysis Testosterone replacement
633
What is Turner's syndrome?
Loss or abnormality of 2nd X chromosome in phenotypic girls 45X
634
Different age presentations of Turners syndrome?
Newborns: borderline small, lymphoedema of hands/feet, excessive skin at nape of neck, cardiac abnormalities Infants: close to third centile, feeding difficulties, poor weight gain, poor sleeping Preschool: short stature, recurrent middle ear infections, behavioural difficulties, high activity levels Adolescence: ovarian failure, impaired pubertal growth spurt, obesity, htn, higher prevalence immune disorders, foot problems, learning disabilities Can have dysmorphic features eg webbed neck, short fingers, lymphoedema, high palate Can have renal and heart problems
635
how to diagnose turner's syndrome?
Via amniocentesis Chromosomal analysis Measure gondatrophins, TFTs, renal and heart tests, bone age
636
Management of turner's syndrome?
MDT Short stature- GH therapy May supplement with oestrogen Gonadal failure-combined oestrogen and progestogen
637
Neonatal features of down's syndrome?
Hyperflexibility, muscular hypotonia, transient myelodysplasia, bradycephaly, oblique palpebral fissures, epicanthic folds, ring or iris speckles (brushfield spots), low set/folded ears, stenotic meatus, flat nasal bridge, protruding tongue, high arched palate, loose skin on nape of neck, single palmar crease, short little finger, short broad hands, gap between hallux and second toes, congenital heart defects, duodenal atresia
638
What is down's syndrome? Biggest risk factor?
trisomy 21 maternal age
639
Management of down's syndrome?
Annual health checks-hearing, resp (OSA), eyes, heart, neurological, GI, hip, thyroid. Extra support in academia and help with living.
640
What is edward's syndrome?
Trisomy 18
641
Presentation of edward's syndrome?
More likely to be female Low birth weight, craniofacial abnormalities (low set and malformed ears, micrognathia, prominent occiput, small facial features, microcephaly, cleft lip), skeletal abnormalities, congenital heart defects, GI abnormalities, GU abnormalities (horse shoe kidney), neurological problems, pulmonary hypoplasia
642
Management of edward's syndrome? What to take into account when making decisions?
Feeding difficulties common, 38.5% foetuses die during labour Chance of survival to 3 months is 20% and to 1 year=8% May decide to not give life sustaining treatment. Otherwise manage cardiac failure and resp insufficiency
643
What is patau syndrome?
Trisomy 13
644
Presentation of patau syndrome?
Many never survive until term and are stillborn or spontaneously abort Low birth weight, congenital heart defects, holoprosencephaly causing midline facial defects, brain and cns abnormalities, GI and GU malformations
645
Life expectancy of patau syndrome?
median is 2.5 days. About 50% live over a week, 5-10% live over a year
646
What is fragile x syndrome?
Most common cause of sex linked general learning disability. Delayed milestones and typical features eg high forehead, large testicles, facial asymmetry, large jaw and long ears.
647
Potential diagnoses coming under fragile x syndrome?
ADHD, ASD, obsessive compulsive behaviours, emotional lability, aggressive behaviours, specific speech disorders eg echolalia and perseveration
648
Management of fragile x patients?
MDT approach and manage each syptom No cure Most diagnosed by age 3 usually
649
Name 4 types of muscular dystrophy?
duchenne muscular dystrophy ( most common and one of most severe) , myotonic dystrophy, facioscapulohumeral muscular dystrophy, becker muscular dystrophy, limb girdle muscular dystrophy, emery-dreifuss muscular dystrophy
650
Male infant not walking by 18 months- concern? what test?
Concerned about duchenne muscular dystrophy measure creatine kinase
651
Presentation of ?muscular dystrophy?
Gower's maneouvre (stands up by facing floor, wide feet, lift bottom first then hands walked up legs) Waddling gait, lordosis/scoliosis, large calves and leg muscles compared to other muscles
652
Management of muscular dystrophy?
Depends on type and severity eg mobility and breathing assistance Steroids Ataluren if has DMD, is over 5 and can still walk Creatine supplements Treat swallow and heart complications
653
What features would make you suspect Angleman's syndrome?
Developmental delay apparent by 6 months, but forward progression with no loss of skills once acquired Sitting late at 12 months, walking late at 3-4 years, 10% can't walk, flat and turned out feet, lean forward when run, speech impairment, laughter and smiling/happy demeanour, excitable personality. hand-flapping, short attention span, microcephaly, epilepsy, sleep disorders, excess chewing, drooling, fascination with water, hypopigmentation, prominent mandible
654
What is the lifespan of angleman's syndrome?
normal lifespan
655
Presentation prader willi syndrome?
Hypotonia, developmental delay, obesity, learning disability, behavioural problems
656
Genetics of angleman's syndrome?
Maternal deletion of chromosome 15 or paternal uniparental disomy
657
Genetics of prader willi syndrome?
Paternal deletion of chromosome 15 or maternal uniparental disomy
658
Diagnostic criteria for prader willi?
Between birth and 3 years: 5 points including 4 major 3 years-adult=8 points including 5 major Major (1 point); hypotonia with poor suck, feeding problems, excessive weight gain 1-6 years, characteristic facies, hypogonadism, cryptorchidism, global developmental delay, hyperphagia, chromosomal abnormality Minor (0.5 points); infantile lethargy, behavioural problems, sleep disturbance, short and failed pubertal growth spurt, hypopigmentation, small hands and feet, narrow hands, eye abnormalities, thick saliva, speech articulation defects, skin picking
659
Management of prader willi?
MDT GH treatment to maintain normal growth SSRIs trial No role for appetite suppressants
660
What is noonan's syndrome (formerly leopard syndrome)?
Autosomal dominant condition characterised by: congenital heart disease, short stature, broad and webbed neck, sternal deformity, developmental delay, facial features, cryptorchidism, increased bleeding tendency
661
What is william's syndrome?
Rare autosomal dominant disease characterised by CV disease, distinctive facies, connective tissue abnormalties, intellectual disability, growth and endocrine abnormalities Specific cognitive profile: good on verbal short term memory and language, but poor visuospatial construction Overly friendly Infantile hypercalcaemia
662
Management of william's syndrome?
MDT management- manage infantile hypercalcaemia with diet and corticosteroids, yearly surveillance, monitoring CV complications
663
What is newborn respiratory distress syndrome?
Also known as hyaline membrane disease and surfactant deficiency lung disease Usually affects premature babies who don't have the necessary surfactant (enough produced by week 34 normally)
664
Presentation of newborn resp distress syndrome?
Peripheral and central cyanosis, rapid and shallow breathing, flaring nostrils, grunting
665
Management of newborn resp distress syndrome?
Treatment during prem labour= steroid injection before delivery and then a 2nd dose 24 hours later Treatment after birth= extra oxygen if mild symptoms. More severe= ventilator, dose of artificial surfactant,IV fluids and nutrition
666
What warrants mild chronic lung disease of prematurity (bronchopulmonary dysplasia)?
Breathing room air at 36 weeks for babies born before 32 weeks or air by 56 days of postnatal age for babies born after 32 weeks
667
What warrants moderate chronic lung disease of prematurity?
Needing less than 30% oxygen at 36 weeks if born before 32 weeks or needing less than 30% oxygen at 56 days of postnatal age if born after 32 weeks
668
What warrants severe chronic lung disease of prematurity?
Need more than 30% oxygen to breathe +/- CPAP at 36weeks/56 days postnatal
669
What can be seen on CXR of a baby with chronic lung disease of prematurity?
Diffuse haziness, coarse interstitial pattern=atelectasis, inflammation, +/-pulmonary oedema
670
Management of chronic lung disease of prematurity?
Minimise ventilation associated lung injury, continuous oxygen monitoring, nasal CPAP, maintain o2 between 91-95%, send them home on oxygen Potential: Dexamethasone short term, diuretics, methylxanthines eg caffeine (increases resp drive and decreases apnoea)
671
What is meconium?
Dark green faecal material produced during pregnancy, which is passed by the baby
672
Why is meconium aspiration bad?
Vasoactive and cytokine substances activate inflammatory pathways and inhibit the effect of surfactant Causes varying degrees of respiratory distress in the newborn Potential consequences: airway obstruction, foetal hypoxia, pulmonary inflammation, infection (chemical pneumonia), surfactant inactivation, persistent pulmonary htn
673
Management of meconium aspiration?
Depends on severity of resp distress eg obs, routine care, ventilation/oxygen, antibiotics, surfactant, inhaled nitric oxide if pulmonary htn, steroids
674
Prognosis of meconium aspiration?
80% have 3-4 day illness then discharged home Complications= air leak, cerebral palsy, chronic lung disease
675
What is hypoxic ischaemic encephalopathy?
also known as intrapartum asphyxia Brain injury caused by oxygen deprivation leading to death or severe impairment eg epilepsy, developmental delay, motor impairment, neurodevelopmental delay and cognitive impairment, cerebral palsy Damage is permanent
676
What events could lead to asphyxia?
Acute maternal hypotension, cardiac complications, injury from umbilical cord, impaired blood flow to brain during birth, intrapartum haemorrhage, placental abruption, labour and delivery stress, trauma etc
677
What comes under TORCH infection?
Toxoplasmosis Others= syphilis, parvovirus b19, vzv, enteroviruses, lymphocytic choriomeningitis virus, HIV, zika Rubella Cytomegalovirus HSV
678
What do torch infections generally do?
Infections during pregnancy that cause congenital defects Generally greater risk of harm if mum infected in early pregnancy Can cause miscarriage or spontaneous abortion
679
Congenital toxoplasmosis issues?
Chorioretinitis with blindness, anaemia, hepatic and neurological symptoms
680
What can the 'others' of Torch infection cause?
Syphilis= bowed sabre shins, hutchinson teeth, typical facial appearance Parvovirus b19= anaemia, hydrops fetalis, myocarditis VZV= chorioretinitis, cataracts, limb atrophy, cerebral cortical atrophy, neurodisability Zika= microcephaly, positional/hearing/ocular abnormalities
681
What can rubella do to the neonate?
Intrauterine growth restriction, intracranial calcifications, microcephaly, cataracts, cardiac defects, neuro disease Blueberry muffin appearance Most occur if exposure during first 16 weeks of pregnancy
682
What can cytomegalovirus do to the neonate?
Growth restriction, sensorineural hearing loss, intracranial calcification, microcephaly, hydrocephalus, hepatosplenomegaly, optic atrophy, delayed psychomotor development More severe in 1st trimester, but more common in 3rd trimester
683
What drug can be used to stop the progression of hearing loss due to CMV?
Ganciclovir
684
Describe physiological jaundice in the neonate?
Increased rbc breakdown and immature liver function Present at 2-3 days of age Jaundice begins to disappear towards end of first week and resolved by 10 days Bilirubin under 200 micromol/L and baby remains well
685
Causes of early neonatal jaundice aka onset in under 24 hours
Haemolytic disease (rhesus, ABO incompatibility, G6PD deficiency, spherocytosis) Infection (toxoplasmosis/TORCH) Haematoma Maternal autoimmune haemolytic anaemia (SLE) Gilbert's syndrome Dubin-Johnson syndrome Crigler-Najjar syndrome
686
Causes of prolonged neonatal jaundice aka lasting over 14 days (term) or over 21 days (preterm)
Infection, hypothyroid, hypopituitarism, galactosaemia, breast milk jaundice, GI eg biliary atresia
687
How common is jaundice in the 1st week of life?
60% term and 80% preterm babies develop it 15% of healthy infants get physiological breastmilk jaundice
688
What is kernicterus?
A complication of neonatal jaundice- bilirubin encephalopathy (unconjugated) Occurs mainly in premature babies.
689
Features of kernicterus?
Early features= jaundice, hypotonia, poor feeding, absent startle reflex May then develop: high pitched cry, hypertonia of extensor muscles with arched back and hyperextended neck, bulging fontanelle, seizures Potential complications= chronic bilirubin encephalopathy, extrapyramidal signs, visual problems, hearing problems, cognitive defects, dental enamel
690
Management of kernicterus?
Manage neurological complications, phototherapy and exchange transfusions
691
What is the most common GI emergency in neonates?
Necrotising enterocolitis
692
What can be found on examination of a baby with necrotising enterocolitis?
Distension, visible intestinal loops, altered stools, bloody mucoid stool and bilious vomiting, decreased bowel sounds, erythema, associated systemic features
693
Management of ?necrotising enterocolitis?
Confirm diagnosis by AXR and get baseline biochemistry Nil by mouth, bowel decompression, Iv fluids and TPN (total parenteral nutrition), IV abx for 10-14 days (gentamicin and metronidazole), surgery if perforated/necrotic bowel suspected NB/ stages i= suspected, iia=mild, iib=moderate, iiia=advanced, iiib=deteriorating
694
What is gastroschisis?
Congenital defect of the abdominal wall where the abdominal contents herniate into the amniotic sack (usually the small intestine, but sometimes stomach, colon and ovaries). There is no covering membrane. The opening is less than 5cm in length
695
Management of gastroschisis?
Scheduled preterm delivery may improve the post op outcome. Primary closure of the defect
696
Simple v complex gastroschisis?
``` Simple= intact, uncompromised and continuous bowel Complex= bowel perforation, atresia or necrosis ```
697
Difference between gastroschisis and exomphalos?
Exomphalos has bowel contents in a sac, is more variable in size, and has a more central location Gastroschisis has no covering membrane, is under 5 cm in length and tends to be to the right of umbilical cord insertion
698
What is oesophageal atresia?
Congenital abnormality with a blind ending oesophagus in isolation or with at least one fistula(e) communicating between normal oesophagus and trachea (tracheo-oesophageal fistula0
699
Associated anomalies with oesophageal atresia?
``` VACTERL syndrome (vertebral, anorectal, cardiovascular, tracheo-oesophageal, oseophageal atreisa, renal abnormalities, limb) CHARGE association (coloboma, heart, atresia, retarded development, genital hypoplasia, ear) Chromosomal abnormalities eg trisomies ```
700
Postnatal presentation of oesophageal atresia?
Respiratory distress, choking, feeding difficulties, frothing in first few hours after birth
701
Management of oesophageal atresia?
Surgery
702
Presentation of bowel atresia?
Persistent vomiting-often bilious and within hours of birth on Xray= double bubble sign of duodenal atresia, colonic atresia has normal air levels
703
Most common metabolic problem in neonates?
Gestational diabetes and hypoglycaemia (glucose passes through placenta and elevates glucose levels in foetus, causing increased insulin secretion, this leads to hypoglycaemia) Plasma glucose under 30 mg/dL
704
Pathophysiology of neonatal hyperthyroidism?
Usually caused by maternal graves disease- antibodies cross the placenta and cause the foetus to develop hyperthyroidism Usually temporary, but can be life threatening.
705
Untreated congenital hyperthyroidism?
Risk of craniosynostosis (early skull closing), intellectual disability, growth failure, hyperactivity in later childhood
706
Management of congenital hyperthyroidism?
Antithyroids and beta blockers- stopped when antibodies have disappeared (usually fully resolved by 6 months) NB/ if mum took thyroid drugs during pregnancy, the child may not show symptoms until 3-7 days
707
How many women carry group b strep in genitals?
1 in 4
708
Presentation of neonate with GBS infection?
Sepsis, pneumonia, meningitis signs, BP changes, seizures
709
Management of GBS infection?
IV antibiotics- penicillin G
710
What can listeria do to a neonate?
Low birth weight, prematurity, circulatory and/or respiratory insufficiency, meningitis, sepsis
711
Management of neonatal listeria infection?
Ampicillin and aminoglycoside eg gentamicin
712
Cause of encephalitis in neonates?
HSV2 from vertical transmission
713
Presentation of HSV encephalitis?
Non specific: decreased levels of consciousness, seizures, lethargy, fever
714
Management of HSV encephalitis? Prognosis?
``` IV aciclovir Highly lethal (around 50%) Complications= deaf, vision loss, cerebral palsy, seizure ```
715
Management of cleft life/palate?
Surgery Cleft lip at 3-6 months Palate at 6-12 months
716
What is ophthalmia neonatorum?
Conjunctivitis in newborn due to vertical transmission (contact with mum's birth canal infected by STI) Commonly chlamydia Also: haemophilus, strep, staph, pseudomonas, HSV, adenovirus, enterovirus
717
Chance of ophthalmia neonatorum in babies born to untreated chlamydia?
30-50%
718
Presentation of ophthalmia neonatorum?
Red, discharging, swollen lids, usually bilateral Lid oedema, conjunctival oedema, mucopurulent conjunctivitis, cornea can be involved
719
Management of ophtalmia neonatorum?
Culture conjunctiva and PCR to establish causative organism ``` Bacterial= systemic penicillin G or cephalosporin for gonorrhoea Chlamydia= systemic erythromycin or topical azithromycin Herpetic= topical and systemic aciclovir ```
720
Premature baby still needing breathing support by 36 weeks corrected age = what?
Chronic lung disease/ bronchopulmonary dysplasia Caused by underdeveloped lungs
721
Management of bronchopulmonary dysplasia?
Oxygen (can further damage lungs), breathing support (CPAP or mechanical ventilation), may need steroids
722
Pathophysiology of retinopathy of premature baby?
Abnormal blood vessel growth are fragile and weak, causing bleeding and leakage Usually bilateral Ranges from abnormal blood vessel growth to full retinal detachment (stage i-v) Can cause blindness
723
Management of retinopathy of premature baby?
Laser therapy or cryotherapy: destroys peripheral retina and slows/reverses abnormal vessel growth, but loses some side vision
724
Why are premature babies at risk of osteopenia?
May not receive the full amount of calcium and phosphorus needed to form strong bones Haven't been as active in the womb and thus have weak bones Very premature babies lose more phosphorus in urine than term babies Can be due to low vit D
725
How to treat osteopenia of premature baby?
Supplements- calcium and phosphorus added to IV fluids or breast milk Special formulas if no breast milk Vit D supplements for babies with liver problems
726
What is periventricular leukomalacia?
White matter surrounding ventricles deprived of blood and thus oxygen. This causes softening of the white matter
727
Consequences of periventricular leukomalacia?
Most commonly cerebral palsy May have visual problems and learning disability Not progressive Presentation depends on amount of brain tissue damaged
728
What is apnoea in a prem baby?
Prem baby pauses breathing for over 15-20 seconds or pauses breathing for under 15 seconds but has bradycardia or low sats Usually self limiting
729
What is transient tachypnoea of the newborn?
Caused by leftover fluid in lungs making it harder for the alveoli to stay open RR over 60 Most improve over 24-48 hours, and most cases self limit
730
Who is more likely to have transient tachypnoea of the newborn?
Born before 38 weeks, c-section delivery, born to diabetic or asthmatic mother, twins, male
731
Forms of child abuse
Physical, neglect, emotional, sexual
732
What may come under child neglect?
Medical eg unimmunised, non attendance Nutritional eg faltering growth, obesity Emotional Educational eg poor school attendance Physical eg inadequate hygiene, persistent infestation, inappropriate clothing Failure to supervise eg frequent A+E trips, injury suggesting lack of care such as sun burn
733
Normal presentation/history of toddler's fracture?
Subtle undisplaced spiral fracture of tibia Usually pre school, sudden twist after an unwitnessed fall Local tenderness over tibial shaft or on general strain on tibia
734
What counts as mild dehydration and features?
Less than 5% Thirst, dry lips, restlessness, irritability
735
What counts as moderate dehydration and features?
5-10% Sunken eyes, decrease skin turgor, decreased urine output
736
What counts as severe dehydration and features?
Over 10% Cold, mottled, hypotensive, anuria, decreased consciousness
737
What fluids needed for not dehydrated/dehydrated/shocked?
Not dehydrated=maintenance Dehydrated=maintenance+deficit Shocked=bolus+maintenance+deficit
738
What maintenance fluids do neonates need?
10% glucose with increasing rate every day Monitor U+Es every day From day 2 if needed= sodium 2-3mmol/kg/day and potassium 1-2mmol/kg/day
739
How to estimate child's weight?
(age + 4) x 2
740
What fluids for maintenance in children? How much?
0.9% NaCl (normal saline) First 10kg=100ml/kg Next 10kg=50ml/kg Every other kg=20ml/kg Calculates how much in 24 hours
741
How to calculate the deficit fluids in children?
Deficit x 10 x wt(kg) Where estimated deficit= mild= negligible, mod=5%, severe=10%
742
What bolus do you use and which volume?
0.9% NaCl, volume=20ml/kg May use 10ml/kg in some cases eg trauma, DKA Tends to be 20ml/kg if shocked in DKA, 10ml/kg if not shocked
743
What constitutes an atypical UTI in children and what do you need to do if this is the case?
``` Atypical= septicaemia/IV abx needed, non e.coli, poor urine flow, abdo/bladder mass, increase creatinine, failure to respond to treatment within 48 hours Recurrent= at least 2 episodes with at least 1 with systemic features, or at least 3 episodes without systemic features ``` Need to investigate for a structural abnormality
744
How to investigate ?structural abnormality in the case of UTI?
1. USS renal tract (looks at size and drainage of kidneys and bladder) 2. Micturating cystourethrogram/MCUG (looks for vesicoureteric reflux, looks at bladder and posterior urethral valve) 3. DMSA scan/radionucleide imaging (looks at renal function and any renal scarring)
745
Features of nephritic syndrome?
Haematuria, proteinuria, impaired GFR, salt and water retention (htn and oedema)
746
Management of nephritic syndrome?
Fluid balance management (input/output, fluid restriction, diuretics, salt restriction) Correct other imbalances (K, acidosis) Dialysis may be needed (uncommon) Penicillin for treatment of an ongoing strep infection
747
Why is sinusitis uncommon in children?
Maxillary sinus exists at birth but only grows to full size after 2nd dentition Ethmomid sinuses only 2-3 cells at birth No/rudimental frontal sinuses at birth (develop by 7-8 years)
748
Narrowest point in child's airway vs adult's?
Children=subglottis Adults=vocal cords
749
What is the most common airway abnormality in children and it's presentation?
Laryngomalacia Normal voice, stridor worse on feeding/exertion/supine, failure to thrive in 10%, increased work of breathing
750
When do you expect primitive reflexes to disappear?
By 4-6 months, lots of babies lose them within 2 months
751
What options for formula for children with cow's milk protein allergy?
Hydrolysed eg althera Amino acid based eg alfamino Goat milk formula from birth Soy formula from 6 months
752
What constitutes faltering growth?
Failure to gain adequate weight or height/length during infancy or early childhood Birth weight \<9th centile with a fall across at least 1 wt centiles Birth weight between 9-91st centile with a fall across at least 2 wt centiles Birth weight over the 91st centile with a fall across at least 3 wt centiles Current weight below 2nd centile whatever the birth weight
753
When would you expect a baby to get back to his birth weight?
3 weeks
754
Causes of osteoporosis in children?
Inherited/congenital eg OI, inborn errors of metabolism, haem problems, idiopathic Acquired eg drug induced eg steroids, endocrinopathies, malabsorption
755
What is osteogenesis imperfecta?
AD inherited condition, v rare Mostly due to defects in type i collaegen Characteristed by bone fragility, fractures, deformity, bone pain, poor growth, impaired mobility
756
How can you manage osteogenesis imperfecta?
MDT management esp ortho team and metabolic bone specialist Bisphophonates
757
Describe the types of osteogenesis imperfecta?
type 1- most common, mild 2- lethal 3- progressively deforming, severe 4- moderate
758
What may indicate a shaken baby?
Hypoxia, subdural haematoma, rib fractures, retinal haemorrhages, may have other fractures, torn frenulum
759
How should a premature baby be fed?
IV fluids/parenteral nutrition as suckle and swallow only starts from 32-34 weeks Start small volumes of expressed breast milk and build up to full feeds slowly (risk of NEC if too quick)
760
Causes of acute scrotum in children?
Testicular torsion!!! Also torsion of appendix testis (self limiting, but if any diagnostic doubt treat as testicular torsion)
761
Vomits and presentation age in pyloric stenosis, malrotation and intussusception
Malrotation: bilious green vomit at 24 hours Pyloric stenosis: milky vomit at 4 weeks Intussusception: milky then green vomit at 6 months

Phase 3A 2021 (7 decks)

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