Paeds 1 Flashcards

1
Q

What is the management of Scarlet fever?

A

Antibiotics

Rest, fluids, good hygiene to prevent spread.

Notify the local PHE centre.

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2
Q

What causes the rash in scarlet fever?

A

the Exotoxin (produces by step pyogenes)

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3
Q

What is the causative organism for Scarlett fever?

A

Streptococcus Pyogenes

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4
Q

What deficiency can cow’s milk cause and how?

A

iron deficiency

  • low content of iron in cow’s milk
  • can cause intestinal blood loss (in 40% infants)
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5
Q

What causes acute epiglottis?

A

Haemophilus B influenza (Hib vaccine protects against this)

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6
Q

Who is immune to parvovirus infection and why?

A

Individuals lacking erythrocyte P antigen

this is because parvovirus replicates in red cell precursors expressing P antigen

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7
Q

Who is at particular risk of Parvovirus infection?

A

a) people with haemoglobinopathies, e.g. SCD - are at greater risk of aplastic crisis with parvovirus infection
b) immunocompromised individuals - can develop chronic infection
c) pregnant women - rarely, parvovirus infection in the first 20 weeks of pregnancy can cause miscarriage or congenital abnormalities if the woman is not immune.

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8
Q

What rash is characteristic of parvovirus infection?

A

bright red cheeks;
can cause a lace-like rash in the trunk and arms, more in women and adults; my itch, partculrarly after a hot bath;

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9
Q

What is hydrops fetalis?

A

a life-threatening condition in which abnormal amounts of fluid accumulate in two or more body areas of an unborn baby. Although the fluid buildup may appear anywhere in the baby’s body, it most often occurs in the abdomen, around the heart or lungs, or under the skin.

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10
Q

standard approach to parvovirus diagosis

A

serology

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11
Q

How is malaria diagnosed?

A

blood film

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12
Q

What does the APTT blood test examine?

A

intrinsic pathway of blood clotting (factors: VIII (and vWF), IX, and XI specifically.

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13
Q

what does aPTT stand for?

A

activated partial thromboplastin time

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14
Q

What alterations in vaccination should be made in patients with haemophilia?

A

stick to the vaccination schedule but give the vaccinations s.c. rather than i.m.

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15
Q

What common medication should be avoided in haemophilia and why?

A

NSAIDs because they can affect platelet function

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16
Q

What is another name for henoch-schonlein purpura?

A

IgA vasculitis

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17
Q

What is the most common vasculitic disease in childhood?

A

Henoch SchΓΆnlein Purpura

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18
Q

Tetrad of HSP

A
  • rash
  • abdominal pain
  • arthritis/arthralgia
  • glomerulonephritis
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19
Q

What are common complications of HSP?

A

Intussusception
Acute renal impairment
Arthritis/arthralgia, typically involving ankles and knees
Pancreatitis

Testicular/scrotal involvement is reported in a minority of cases in boys.

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20
Q

Why do you have to hyperhydrate patents with high WCC leukemias?

A

TLS is a risk; TLS can progress to renal failure;

Therefore hyper hydration and allopurinol should be commenced immediately.

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21
Q

Levels of what can be increased in cancers with high cell numbers or a high mass and why?

A

potassium, phosphate and nuclear debris (causing high uric acid and LDH

Any cancer with a high number of cells or a high mass, will undergo lysis resulting in release of these.

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22
Q

First line management of acute asthma?

A
  1. high flow oxygen (10-15L/min via non-rebreather mask)
  2. inhaled salbutamol
    5mg (2.5 mg in children under 5) in 4ml 0.9% NaCl with 6-8 L/min O2.
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23
Q

What is HSP often preceded by?

A

viral URTI

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24
Q

What % of meconium ileus are associated with CF?

A

90%

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25
Why is uric acid high in TLS?
Due to breakdown of genetic material
26
Management of TLS
hyperhydration crystalloid (0.9% NaCl with 5% dextrose) -> no K+ because levels are high in TLS allopurinol
27
complication of TLS
renal failure due to phosphate and uric acid precipitating in the tubules.
28
Why do you hyperhydrate in TLS?
to dilute the phosphate and potassium in the blood also to make sure the kidneys are perfused?
29
What fluid to you give in TLS to hyperhydrate in paediatrics?
Crystalloid: 0.9% NaCl with 5% dextrose to ensure constant avoid potassium in the bag because in TLS the levels of potassium are high give fluids according to weight
30
What does allopurinol do and how?
decreases level of uric acid by inhibiting XO oxidase which is involved in the breakdown of purines into uric acid
31
What does allopurinol do and how?
decreases level of uric acid by inhibiting XO oxidase which is involved in the breakdown of purines into uric acid (pg 42 top drugs)
32
Hyperleucocytosis
thick blood due to high number of white cells here be careful when transfusing e.g. platelets because you do not want to make the blood more viscous; only give small amounts.
33
Paedatric oncological tertiary centres in London
north: GOSH for young children, UCLH for approx above 13 south: royal Marsden
34
Febrile neutropenia
anyone with Neutrophils <0.5 and >38 RISK: (gram -ve) neutropenic sepsis
35
Why do children with Leukemia get Cotrimoxazole every week?
to prevent opportunistic PCP
36
What do you give to prevent line infection?
teicoplanin (against gam +ve)
37
What systems does HSP usually affect?
skin, GI tract, renal, joints
38
typical joint problems in HSP
bilateral large join swelling (ankle, knee, wrist) -> treat with NSAID eg ibuprofen
39
urine dipstick in HSP dipstick renal failure? what other tests to do?
(microscopic) haematuria and proteinuria Cr, UREA, BP
40
Where in London are children with haemophilia treated?
GOSH when grow up: royal free
41
What are common indications for allopurinol?
1. prevent recurrent attacks of gout 2. prevention of uric acid and calcium oxalate real stones 3. to prevent hyperuricaemia and TLS ass. with chemotherapy
42
MOA allopurnol
xanthine oxidase inhibitor
43
What are the most common childhood cancers in the UK?
1. Leukemia (32%) 2. Brain and spinal tumours (24%) 3. Lymphomas (10%) 4. Neuroblastoma (7%) 5. Soft tissue sarcoma (7%) 6. Wilms tumour (6%) 7. Bone tumours (4%) 8. Retinoblastoma (3%) 9. Other (7%)
44
Example of an inherited childhood cancer (incl genetic mutation)
bilateral retinoblastoma (mutation within RB ? chromosome 13)
45
What cancers is Down syndrome associated with?
leukemia neurofibromatosis glioma
46
What are the most common childhood cancers in Africa?
Burkitts lymphoma and kaposis sarcoma
47
what are examples of infection related cancers and what pathogens are they associated with?
Burkitt's lymphoma, hodgkin lymphoma, nasopharyngeal carcinoma - EBV liver carcinoma - hep B Kaposis sarcoma - HIV and human herpesvirus 8
47
what are examples of infection related cancers and what pathogens are they associated with?
Burkitt's lymphoma, hodgkin lymphoma, nasopharyngeal carcinoma - EBV liver carcinoma - hep B Kaposis sarcoma - HIV and human herpesvirus 8
48
levels of what in urine can be useful in confirming the diagnosis of neuroblastoma?
increased urinary catecholamine excretion (VMA - vanillylmandelic acid; HVA - homovanillic acid)
49
When is a-fetoprotein increased?
in liver tumours and in germ cell tumours
50
What translocation in seen in Ewing sarcoma?
chromosomes 11 and 22
51
Cancer survival of teenagers+young adults vs children
teenagers and young adults have poorer outcomes
52
what do ALL and CLL stand for?
acute lymphoblastic leukemia and chronic lymphocytic leukemia
53
What is the problem with radiotherapy in children?
- the risk of damage to growth and function of normal tissue is greater in a child than in an adult - practical difficulties due to the need for adequate protection of normal tissues, careful positioning and immobilisation of the patient during treatment - cranial radiotherapy in children <3yo -> significant risk of severe damage to neurocognitive development
54
Summarise the important short term side effects of chemotherapy in children
BM suppression -> anaemia, thrombocytopenia and bleeding, neutropenia (-> infection) Immunosuppression -> Infection Gut mucosal damage -> infection (particularly gram-ve) and undernutrition, diarrhoea N&V -> undernutrition anorexia -> undernutrition alopecia
55
What to do if a child presents with fever and neutropenia?
admit promptly to hospital for cultures and treatment with broad-spectrum antibiotics
56
What are some opportunistic infections commonly seen in children that are immunocompromised due to cancer (therapy)?
- Pneumocystis jiroveci (carinii) pneumonia (especially in children with leukemias - disseminated fungal infection e.g. aspergillosis and candidiasis - coagulase-negative staphylococcal infections of central venous catheters
57
What vaccines are contraindicated during and following cancer treatment and how long?
live vaccines are CI'd during chemotherapy and for 6-12 months subsequently due to depressed immunity. After this period re-immunisation against common childhood infections is recommended.
58
What viral infections are dangerous for children undergoing chemotherapy?
- Measles - Varicella Zoster -> may have atypical presentation and may be life-threatening If at risk of contact with measles or chickenpox, some protection can be afforded by prompt administration of immunoglobulin or zoster immune globulin. Aciclovir is used to treat established varicella infection but no treatment is available for measles. Most other viral infections are no worse than in other children.
59
What are the options for venous access in chemotherapy?
-tunnelled venous catheter (Hickman lines) - implantable ports (=left entirely under the skin)\\central venous catheters can remain in situ for many months if not years but carry the risk of infection and can get blocked or split.
59
What are the options for venous access in chemotherapy?
-tunnelled venous catheter (Hickman lines) - implantable ports (=left entirely under the skin) central venous catheters can remain in situ for many months if not years but carry the risk of infection and can get blocked or split.
60
What is the peak age of ALL presentation?
2-5
61
What is the commonest leukemia in children?
ALL -> makes up 80% of all childhood leukemias followed by AML and ANLL (acute nonlymphocytic leukemia)
62
Why are steroids given when treating leukemia?
x
63
Treatment of leukemia
1. make sure anaemia, thrombocytopenia and TLS are sufficiently under control before starting chemotherapy 2. remission induction with combination chemotherapy including steroids 3. Intensification: a block of intensive chemotherapy is given to consolidate remission (better cure rates but higher toxicity) 4. CNS - cytotoxic drugs poorly penetrate the CNS; leukaemia cells at this site may survive effective treatment, therefore intrathecal chemotherapy is given to prevent CNS relapse; patients with CNS disease evidence at presentation are given additional doses of intrathecal chemotherapy during induction. 5. continuing therapy at modest intensity for a relatively long time period up to 3 years from dx; cotrimoxazole prophylaxis is given to prevent PCP
64
What is given to prevent PCP in leukemia?
prophylactic cotrimoxazole
65
What is co-trimoxazole and what is it used for?
Trimiethoprim with sulfonamide e.g. sulfamethoazole prevention of PCP in immunosuppression, e.g. HIV or leukemia or chemotherapy (trimethoprim ought to have broad activity against gram +ve and -ve bacteria; combination with sulphonamide extends the spectrum to include activity against the fungus causing PCP
66
What are the most common solid tumours in children?
brain tumours
67
What is different about brain tumours in children compared to adulrs
in children they are almost always primary rather than metastatic and 60% are infratentorial (located below the tentorium cerebelli)
68
What is the leading cause of childhood cancer deaths?
brain tumours
69
Liste the types of brain tumours in children
- astrocytoma (40%) - varies from benign to highly malignant (glioblastoma multiforme) - medulloblastoma (20%) - arises from the midline of the posterior fossa - ependymoma (8%) - mostly in posterior fossa where it behaves like medulloblastoma - brainstem glioma (6%) - malignant tumours associated with a very poor prognosis - craniopharyngioma (4%) - developmental tumour arising from the squamous remnant of Rathke pouch; not truly malignant but locally invasive. - atypical teratoid/rhabdoid tumour - a rare type of aggressive tumour that most commonly occurs in young children
70
Which lymphomas are more common in childhood and which in adolescence?
NHL is more common in childhood; Hodgkin lymphoma is more common in adolescence.
71
What causes neonatal respiratory distress syndrome?
lack of surfactant
72
where do neuroblastoma and related tumours arise from?
neural crest tissue in the adrenal medulla and sympathetic NS
73
how does neuroblastoma present?
most children have an abdominal mass, but the primary tumour can lie anywhere along the sympathetic chain from the neck to the pelvis; classically the abdominal primary is of adrenal origin but at presentation the tumour mass is often large and complex, crossing the midline, enveloping major blood vessels and lymph nodes.
74
At what age is neuroblastoma most common?
Under the age of 5
75
How is neuroblastoma diagnosed?
- characteristic clinical and radiological features - increased levels of urinary catecholamines - confirmatory biopsy - evidence of metastatic disease with BM sampling and MIBG scan
76
Management of neuroblastoma
- localised primaries without metastatic disease can often be cured with surgery alone - in some infants neuroblastoma (incl. metastatic) may resolve spontaneously - metastatic disease in older children is treated with chemotherapy (incl. high dose therapy with autologous stem cell rescue, surgery and radiotherapy) high risk of relapse
77
Where does Wilms tumour originate from?
embryonal renal tissue
78
what is the most common renal tumour of childhood?
Wilms tumour
79
In what age groups is Wilms tumour seen?
over 80% present before 5yo and it is rarely seen after 10yo
80
Clinical presentation and features of Wilms tumour
most children present with large abdominal mass (and are otherwise well) common: abdo mass, haematuria uncommon: abdo pain, anorexia, anaemia (haemorrhage into mass), hypertension
81
Where does Wilms tumour normally metastasise to?
lungs
82
Investigations for Wilms tumour
US +/- CT/MRI usually characteristic with intrinsic renal mass distorting normal structure. staging to assess distant mets, initial tumour respectability and function of contralateral kidney
83
Management of Wilms tumour
1. initial chemotherapy 2. delayed nephrectomy -> histological staging, surgical and path findings helps plan further management 3. radiotherapy in more advanced disease in bilateral disease (5%) particularly careful management to preserve as much renal function as possible
84
What % children with Wilms tumour present with bilateral disease?
5%
85
prognosis of Wilms tumour
good >80% pts cured with metastatic disease (15%) the cure rate is >60% relapse carries a poor prognosis
86
What is the most common soft tissue sarcoma in childhood?
Rhabdomyosarcoma
87
What tissue does rhabdomyosarcoma originate from?
primitive mesenchymal tissue
88
Are bone tumours in children more common in girls or boys?
boys
89
are malignant bone tumours more common before or after puberty?
after
90
what is more common - osteosarcoma or Ewing sarcoma?
osteosarcoma is more common but Ewing sarcoma is more common in younger children. Ewing sarcoma is the second most common
90
what is more common - osteosarcoma or Ewing sarcoma?
osteosarcoma is more common but Ewing sarcoma is more common in younger children.
91
Clinical presentation of bone tumours
persistent, localised bone pain -> indication for early x-ray. at dx most patients are otherwise well.
92
investigations in bone tumours
plain x-ray -> MRI -> bone scan Chest CT to asses for lung mets and BM sampling to exclude marrow involvement
93
Management of bone tumours in children
1. combination chemotherapy 2. surgery: avoid amputation wherever possible with en bloc resection of tumours with end-prosthetic resection radiotherapy in Ewing sarcoma in local disease, especially if surgical resection is impossible or incomplete e.g in pelvis or axial skeleton.
94
Define retinoblastoma
rare, malignant tumour of retinal cells accounts for 5% of visual impairment in children; may affect one or both eyes.
95
What % of bilateral and unilateral retinoblastoma tumours are hereditary?
100% of bilateral 20% of unilateral
96
On which chromosome is the retinoblastoma susceptibility gene? What is the inheritance pattern?
13 dominant (but with incomplete penetrance)
97
When do pts with retinoblastoma usually present?
usually in the first 3 years of life
98
clinical features of retinoblastoma
white pupillary reflex
99
investigations for retinoblastoma
MRI and examination under anaesthetic; tumours are frequently multifocal. no biopsy is done, mx based on ophthalmological findings
100
management of retinoblastoma
aim: cure yet preserve vision - enucleation of the eye may be necessary for advanced disease - chemotherapy (particularly in bilateral disease) to shrink tumour followed by local laster treatment to the retina - radiotherapy may be used in more advanced disease but is usually reserved for recurrence. most patients are cured but many visually impaired.
101
How do children usually present with Kaposis sarcoma? How is it diagnosed and treated?
generalised lymphadenopathy dx requires biopsy confirmation Mx with chemotherapy and ART triggered by HHV-8
102
What cells is kaposi's sarcoma derived from?
blood and lymph vessel cells
103
What are the common childhood cancers according to age groups?
Pre-school (<5) - ALL (peak incidence) - NHL - neuroblastoma - wilms tumour - retinoblastoma School-aged - ALL - brain tumours Adolescnece - ALL - hodgkin lymphoma - malignant bone tumours - soft tissue sarcomas
104
What part of the brain is affected in cerebral palsy?
cerebral cortex (motor cortex in particular) and other components of the brain involved in strength, movement and muscle tone.
105
What are the patterns of cerebral palsy?
- increased tone(hypertonia, usually spasticity) - decreased tone also possible (hypotonia, atonia) - dysregulated movement (ataxia) - altered movements (dyskinesia) - combination/mixed pattern
106
What types of cells are UMNs usually?
pyramidal cells
107
What happens with muscle tone in UMN and LMN lesions?
UMN: hypertonia LMN: hypotonia
108
What is the relationship of UMN and cerebral palsy?
alteration of UMN physiology can result in impaired volitional motor control and loss of UMN's inhibitory effects on LMN. -> loss of 'braking' action of UMN in LMN -> impairment of normal movement initiation -> impairment of smooth recovery of motor action Predominance of uninhibited LMN function in cerebral palsy resulting in spasticity/increased tone, weakness, and hyperreflexia.
109
What proportion of CP are due to hypoxic-ischaemic encephalopathy (HIE)?
10-20%
109
What proportion of CP are due to hypoxic-ischaemic encephalopathy (HIE)?
10-20%
110
What are the indications to prescribing steroids?
1. treatment of allergic or inflammatory disorders e.g. anaphylaxis or asthma 2. treatment of autoimmune disease e.g. IBD or inflammatory arthritis 3. cancer treatment (as part of chemotherapy or to reduce tumour-related swelling) 4. hormone replacement in adrenal insufficiency or hypopituitarism
111
MOA of steroids
- bind to ic GC recentres, alter gene expression - increase anti-inflammatory actions - reduce pro-inflammatory actions such as cytokines and TNF-alpha mainly GC effects. MC effects are increased retention of Na+ and water and excretion of K+ in the renal tubules
112
What are ossification centres?
areas where bone tissue develops
113
In what order do secondary ossification centres in the elbow develop and at what ages?
CRITOL / CRITOE capitulum radial head internal (medial) epicondyle trochlea olecranon lateral/external epicondyle 1,3,5,7,9,11 or 1, 5,7,10,10,11 (more accurate)
114
What are the different parts of long bones and where are growth plates found?
diaphysis - shaft metaphysis - between d and e epiphysis - at the end of the bone growth plate is between m and e
115
How can you classify epiphyseal fractures?
Salter Harris I - epiphyseal slip only II - fracture through epiphyseal plate with triangle of shaft attached III - fracture through epiphysis extending into epiphyseal plate IV - fracture of epiphysis and shaft, crossing the epiphyseal plate V - crush injury (see image on google)
116
Considerations for terminating continuous steroid therapy?
sudden withdrawal can lead to Addisonian crisis with cardiovascular collapse. This is because exogenous steroids suppress pituitary ACTH, switching off the stimulus for corticosteroid production and lead to adrenal atrophy. Withdraw slowly for gradual return off adrenal function.
117
Symptoms of chronic glucocorticoid deficiency on exogenous steroid withdrrawl
fatigue arthralgia weight loss
118
SE of glucocorticoids
- Immunosuppression - diabetes mellitus - osteoporosis - proximal muscle weakness - skin thinning - easy bruising - gastritis - mood and behavioural changes (insomnia, confusion, psychosis, suicidal ideas) - hypertension - hypokalaemia - oedema
119
In which groups of people should you be careful when giving steroids?
- in people with infection - in children (in whom it can suppress growth)
120
Name important interactions of steriods
- NSAIDs: in combination, increase the risk of peptic ulceration and GI bleeding - b2-agonists, theophylline, loop or thiazide diuretics: enhance hypokalaemia - efficacy reduced by cytochrome p450 inducers (phenytoin, carbamazepine, rifampicin) - vaccines: corticosteroids reduce immune response to them
121
how should OD steroids be taken?
in the morning to mimic the circadian rhythm and reduce insomnia
122
What advice should you give patients on steroid replacement therapy?
Double the dose when feeling ill, reduce back to maintenance dose on recovery
123
Which steroids are most potent?
Dexamethasone (no MC activity) Fludrocortisone methylprednisolone Prednisone/Prednisolone Hydrocortisone
124
what medications may need to be co-prescribed in steroid therapy?
PPIs and bisphosphonates to limit adverse effects
125
What is in the 6in1 vaccine?
diphtheria, tetanus, pertussis (whooping cough), Hib, Hep B, polio
126
What is in the 4in1 vaccine and when is it given?
pre-school booster diphtheria tetanus polio whooping cough/pertussis
127
What is in the 3in1 vaccine and when is it given/
diphtheria tetanus polio teenage booster (14 yo)
128
What vaccines are given at 14 years?
3in1 MenACWY
129
What vaccinations are given at 2 years?
Hib/MenC MMR PCV (pneumococcal) MenB
130
What vaccines are given at 3 years and 4 months?
4in1 pre-school booster (Diphtheria, Tetanus, Polio, pertussis) MMR (2nd dose)
131
What vaccines are given at 1 year?
Hib/MenC (1st dose) MMR (1st dose) Pneumococcal (PCV) vaccine (2nd dose) MenB (3rd dose)
132
What vaccines are given before 1 year of age?
8 weeks 6-in-1 vaccine Rotavirus vaccine MenB 12 weeks 6-in-1 vaccine (2nd dose) Pneumococcal (PCV) vaccine Rotavirus vaccine (2nd dose) 16 weeks 6-in-1 vaccine (3rd dose) MenB (2nd dose)
133
What is the Abx of choice in acute otitis media?
amoxicillin
134
What are the indications for prescribing antibiotics (amoxicillin) in otitis media in children?
Eardrum is perforated < 2 years old and bilateral infection Present for β‰₯4 days < 3 months old
135
CREAM acronym for kawasaki disease
C - conjunctivitis R - rash E - edema/eryhthema of hands A - adenopathy (most commonly cervical, usually unilateral) M - mucosal involvement (strawberry tongue, oral fissures etc) Fever!!
136
What is tetralogy of Fallot?
Tetralogy of Fallot is a relatively rare form of congenital cardiac disease. It is cyanotic. - Ventricular septal defect (VSD) - Overriding aorta - i.e., an aorta that overrides the right and left ventricles as a result of the VSD - Right ventricular outflow tract obstruction (RVOTO) - this is the main determinant of the severity of cyanosis - Right ventricular hypertrophy
137
How do you calculate maintenance fluids required in children?
1st 10kg of bodyweight at 100ml/kg/day 2nd 10kg of bodyweight at 50ml/kg/day Remaining bodyweight at 20ml/kg/day The fluid type routinely used is 0.9% sodium chloride + 5% dextrose. Potassim is added as required depending on their U&Es.
138
What gastro condition is prematurity a RF for and what is a typical radiological finding suggestive of this condition?
Necrotising Enterocolitis pneumatosis intestinalis on abdo X-ray
139
What is montelukast?
Monteleukast is an LTRA (leukotriene receptor antagonists) which works by downregulating inflammatory leukotrienes, and can be used an add-on therapy.
140
What is the drug known as Ritalin and what is it used for?
Methylphenidate used for ADHD
141
What is the formula for calculating height velocity?
(height now - height last visit) / (age now - age last visit) expressed in cm/year measured at 6 month intervals
142
What controls growth?
GH - IGF-1 axis
143
What type of hormone is GH? (biochem)
single polypeptide
144
Which cells release growth hormone?
somatotrophs in the anterior pituitary gland
145
In what manner is GH released and what influences its release?
pulsatile sleep, exercise, stress, nutrition, age (children produce more than adults)
146
When is the most rapid phase of growth?
antenatal
147
What factors influence antenatal growth?
maternal health placenta
148
Describe the growth within the first year of life?
it is a continuation of fetal growth mainly dependent on nutrition, rather than GH (GH influences growth at 9-12 months) baby grows 23-25 cm in first year (rapid initial growth)
149
Describe growth in early childhood (post infancy to adolescence)
- similar growth rates in boys and girls - GH/IGF-1 drives growth - nutrition less impact
150
When should you investigate a Childs health when growth problems are suspected?
- are they too short/tall for their age? - have they jumped across centiles? - has puberty started and is it progressing normally? - is growth normal fo stage of puberty? - is the child overweight or obese?
151
What are causes of short stature?
- genetics (i.e. short parents) - pubertal and growth delay - IUGR/SGA (not all catch up) - dysmorphic syndromes (e.g. Down syndrome) - endocrine disorders (cushings, hypothyroid, GH def) - chronic paediatric disease - psychosocial deprivation
152
What questions do you ask when a child with short stature presents?
- parents heights (-> mid parental centime) - birth history and weight (IUGR?) - medical history - are there previous measurements?
153
What is the mid parental centile and how is it calculated?
What the 50th centile would be for that family boys: (moms height + dads height)/2 +7 girls: (moms height + dads height)/2 -7
154
Investigations for a child that is falling in centiles
FBC CRP Liver function kidney function thyroid function ferritin / serum iron coeliac screen IGF-1 bone age
155
What syndromes can lead to abnormal growth?
Turners (XO) Down's syndrome skeletal dysplasias
156
Why can significant illness impact growth?
inflammation poor nutrition side effects of drugs such as steroids
157
Does surgical confirmation of testicular torsion indicate the need for uni- or bilateral orchidopexy (fixation)?
bilateral
158
Is an undescended testis a RF for testicular cancer in only the undescended testis?
In undescended testis, both testes are at risks of tumour development.
159
What do classical molloscum contagious lessons look like?
The lesions are raised papules with central dimpling of lesions.
160
What pathogen causes molloscum contagiousum?
Molluscum contagiosum is caused by a DNA pox virus, specifically a member of the Poxviridae family
161
How do you treat molloscum contagiosum?
Reassurance - benign lesions arising from a poxvirus infection - harmless - but cause a lot of distress to parents and child - condition lasts around a year and then spontaneously resolves usually without any scarring. - presence of extensive mollusca in adults raises the possibility of an immunodeficiency state such as HIV infection and an appropriate history and investigation should be instigated.
162
Why is measles such a problem
It is EXTREMELY contagious. Not that fatal, but it spreads so fast that it can still cause many deaths (as %of people with infection deteriorating) because of how many people get infected so absolute numbers can be high.
163
When treating DKA in children, what is the correct insulin infusion dosage?
0.05 and 0.1 units/kg/hour glucose boluses are no longer recommendable for treatment of paediatric DKA
164
is intussusception more common in males or females?
males (2:1)
165
What is the classical triad of symptoms in intussusception?
vomiting, bloody stools and abdominal pain (colic).
166
At what age is intussusception most common?
5-12 months-of-age but can occur in any age
167
When should colic be taken more seriously?
Colic is a non-specific symptom. β€œsimple” colic will have resolved within the first 3 months of life A presentation like this after 3 months has to be taken more seriously (can be e.g. intussusception)
168
If a mass is palpable in intussusception where is it most often found?
RUQ
169
What is the most common type of intussusception?
ileo-colic (=ilio-caecal) 90% A portion of terminal ileum intussuscepts through the ileo-caecal valve into the colon. It may sometimes extend all the way to the rectum. Other rarer types of intussusception include ileo-ileal, colo-colic, and ileo-ileocolic. The majority of intussusceptions are idiopathic.
170
Which three actions should be the immediate management of a patient with intussusception?
Resusitation radiological air reduction inform surgical team
171
How does radiological air reduction for intussusception work?
Air is introduced under pressure through a rectal catheter. A manometer monitors the pressure and this should not exceed 110mmHg. The air pressure meeting the intussusception usually forces the inverted bowel back through the ileo-caecal valve and into its expected position. This is visualised under fluoroscopic (X-ray) control
172
Conditions associated with intussusceptions
Henoch-Schonlein purpura (intestinal wall haematoma) cystic fibrosis (hypertrophied mucosal glands) and lymphoma In younger infants (the most common group), it is hypothesized that hypertrophied Peyer’s patches (enlarged mesenteric lymph nodes) following a respiratory infection or gastroenteritis may serve as the lead point.
173
What is HUS a triad of?
Thrombocytopenia Microangiopathic haemolytic anaemia Acute renal failure
174
What is the commonest trigger for HUS?
Escherichia coli 0157:H7 serotype causing bloody diarrhoea
175
What pathogens can cause bloody diarrhoea?
bacteria rather than viruses e.g. E. coli or salmonella
176
What is the common blood gas pattern seen in pyloric stenosis?
hypochloraemic, hypokalaemic metabolic alkalosis.
177
What procedure is used to correct pyloric stenosis?
Ramstedt’s pyloromyotomy
178
How common is pyloric stenosis?
occurs in 0.3% births
179
What does red current jelly stool mean?
late stage of intussuscepttion
180
Where does the QT interval start and end, how do you calculate it and what is a normal range?
from start of Q wave until end of T-wave. QT = QT/sqrt(RR) Bazett formula
181
Normal Resp rates in children
Neonate: 30-50 (>60 is tachypnoeic) Infants: 25-40 (>50 is tachypnoeic) Young children: 25-35 (>40 is tachypnoeic) Older children: 20-25 (>3Β§0 is tachypnoeic
182
What are signs of increased work of breathing?
- Nasal flaring - expiratory grunting (to increase positive end-expiratory pressure) - use of accessory muscles, esp. sternomastoids - retraction (recession) of the chest wall, from use of suprasternal, intercostal, and subcostal muscles - difficulty speaking (or feeding)
183
features of cardiac failure in children
- poor feeding/faltering growth - sweating - tachypnoea - tachycardia - gallop rhythm - cardiomegaly - hepatomegaly
184
features of significant murmurs
185
What might you find on examination of the chest wall in children?
- hyper expansion or barrel shape e.g. in asthma - pectus excavatum or pectus carinatum
186
features of cardiac failure in children
- poor feeding/faltering growth - sweating - tachypnoea - tachycardia - gallop rhythm - cardiomegaly - hepatomegaly
187
features of significant murmurs
- conducted all over the precordium - loud - thrill (equals grade 4-6 murmur) - any diastolic murmur - accompanied by other cardiac signs
188
Management of Kawasaki disease?
High dose aspirin and IVIG (within 10days of onset)
189
Adrenaline doses for anaphylaxis in different age groups
1:1000 IM Adrenaline adult: 500 microgramm (0.5ml) 12+ : 500 microgramm (0.5ml) 6-12 : 300 microgramm (0.3ml) <6 : 150 microgramm (0.15ml) repeat after 5 min if not better
190
Define Primary nocturnal enuresis
where children continue to wet the bed past the age of 5.
191
define Perthes disease
Perthes disease is avascular necrosis of the femoral head in children aged 4-8. Disruption of blood flow to the femoral head causes ischemia.
192
Epidemmiology of perthes disease
Perthes disease is 5 times more common in boys, and 10% of cases are bilateral.
193
What is fragile X syndrome and what is the underlying abnormality?
Fragile X syndrome is a genetic condition caused by a CGG trinucleotide repeat in the FMR1 (fragile X mental retardation 1) gene on the X chromosome, affecting synaptic development. Fragile X syndrome is the most common inherited cause of learning disabilities.
194
Causes of stridor in children
- croup - laryngomalacia - foreign object inhalation - epiglotittis
195
Interpreting APGAR scores
A score of >7 is reassuring A score of 4-6 requires stimulation A score of <4 requires resuscitation
196
What is the Paul Bunnell test used to diagnose?
infective mononucleosis
197
What antibiotics are in co-amoxclav?
amoxicillin and clavulanic acid
198
What is the carrier rate of the CF gene in caucasians?
1 in 25
199
What is the life expectancy of people newly diagnosed with CF?
50-55 years
200
Management of HIE
- Resp support - anticonvulsants (for clinical seizures) - hypothermia (if moderate or severe) - fluid restriction b/c of transient renal impairment - Mx of hypotension by volume and inotrope support - monitoring and treatment of hypoglycaemia, electrolyte imbalance (esp. hypocalcaemia)
201
What is Erb Palsy?
- brachial plexus damage during birth at C5 and C6 - Waiters tip position - occur at breech deliveries or with shoulder dystocia - usually resolves completely, if not by 2-3 months refer to plastic surgery - may be accompanied by phrenic nerve palsy -> elevated diaphragm
202
What is surfactant?
- secreted by type II pneumocytes - contains protein and phospholipids - keeps surface tension in alveoli
203
What does a lack of surfactant lead to?
- decreased surface tension - alveolar collapse and inadequate gas exchange Respiratory distress Syndrome
204
What to pregnant women receive when preterm delivery is expected and why?
Glucocorticoids because they stimulate surfactant poduction
205
What is the fluid requirement of a preterm infant on the first day of life?
60-90 mls/kg
206
Why are premature babies at higher risk of iron deficiency?
- low iron stress (iron is mostly transferred to the foetus during the third trimester) - frequent blood sampling with inadequate erythropoietin response
207
Why are preterm babies at higher risk of infection?
- IgG is mostly transferred during the 3rd trimester - infection around cervix is often reason for premature labour and can cause infection soon after birth - infections ass. with caterers, mechanical ventilation etc.
208
mortality of necrotising enteocolitis
20%
209
What is the bowel of a premature infant vulnerable to and what disease can this cause?
- ischaemic injury - bacterial invasion -> necrotising enteocolitis
210
Management of necrotising enterocolitis
- stop oral feed - broad spectrum ABx to cover aerobic and anaerobic organisms - parenteral nutrition - mechanical ventilation - circulatory support - surgery for bowel perforation if needed
211
What maternal condition is congenital heart block associated with?
SLE
212
What is another term for glioblastoma multiform?
grade 4 astrocytoma
213
Which type of bilirubin is raised in physiological jaundice in newborns?
unconjugated
214
Which bilirubin is elevated in physiological Jaundice?
unconjugated -> presence of foetal erythrocytes (lifespan 70 not 120 days, higher volume, higher turnover than adults; higher levels of bilirubin to break down; high Hb concentration at birth) -> immature liver -> less efficient u-bilirubin to c-bilirubin metabolism for excretion into GI tract
215
Measles causative organism
Measles paramyxovirus
216
Which virus causes chicken pox?
HHV3 = varicella virus
217
Which virus causes infectious mononucleosis?
EBV = HHV4
218
What is given to children that were born pre-term prophylactically to prevent lower resp tract disease caused by RSV?
Palivizumab monoclonal antibody that targets the fusion (F) glycoprotein on the surface of RSV
219
What % of newborn infants become visibly janundiced?
50%
220
Why do we treat neonatal jaundice?
unconjugarted bilirubin can be deposited in the brain (particularly basal ganglia), causing kernicterus.
221
what is kernicterus?
Encephalopathy resulting from the deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei it may occur when the level of unconjugated bilirubin exceeds the albumin binding capacity of bilirubin in the blood. free bilirubin is fat soluble and can therefore cross the BBB causing neurotoxic effects fro transient disturbance to severe damage and death.
222
In which infants does Kernicterus still occur?
slightly preterm infants (35-37w) and dark-skin tined infants in whom jaundice is more difficult to detect.
223
Why is Kernicterus more rare? What used to be a common cause?
Kernicterus used to be an important cause of brain damage in infants with severe Rhesus haemolytic disease, but has now become rare since the introdution of prophylactic anti-D Ig for Rh- mothers.
224
What causes jaundice at <24h?
Haemolysis (Rhesus incompatibility; ABO incompatibility; G6PD deficiency; spherocytosis; pyruvate kinase deficiency)
225
What is Crigler-Najar syndrome?
- rare genetic disorder (incidence: 1 in 750,000-1,000,000) - glucuronyl transferase deficiency/absence - results in extremely high levels of unconjugated bilirubin
226
How does phototherapy work?
light at 450nm (blue-green band of visible spectrum) converts unconjugated bilirubin into a harmless water soluble pigment that is predominantly excreted via the urine.
227
How does exchange transfusion for jaundice work?
blood is removed from the baby in small aliquots and replaced with donor blood usually 2x the infants blood volume is exchanged (2x90ml/kg)
228
Why are soft cheeses, undercooked poultry and unpasteurised milk dangerous in pregnanct women?
may lead to listeria monocytogenes infection which can cause - spontaneous abortion - preterm delivery - fetal/neonatal sepsis
229
What should be done in women with genital herpes lesions at time of delivery?
deliver baby via c-section to prevent transmission they baby
230
What should be done if mum has Hep B?
vaccinate baby against HepB shortly after birth to prevent horizontal transmission
231
Symptoms of Hypoglycaemia in babies
- bitterness - irritability - apnoea - lethargy - drowsiness - seizures
232
Commonest cause of perinatal stroke?
ischaemia of the MCA
233
What imaging confirms perinatal stroke?
MRI
234
What is the difference in presentation of HIE and perinatal stroke?
in perinatal stroke you get isolated seizures (=without abnormal clinical features)
235
Incidence of oesophageal atresia
1 in 3500 live births
236
What can babies presenting with duodenal atresia also have?
VACTERL assocation
237
What are the components of the VACTERL association?
V - vertebral anomalies A - anorectal C - cardiac TE - tracheo-oesophageal R - renal L- radial/limb anomalies
238
What are the symptoms of PDA?
- apnoea - bradycardia - increased O2 requirement - difficulty weaning off ventilation increased PP, bounding pulse, may have systolic murmur may also cause no symptoms at all.
239
What is the Pierre robin sequence?
Association of - micrognathia - posterior displacement of the tongue (glossoptosis) - midline cleft of the soft palate
240
Causes for SBO in neonates
- atresia or stenosis of the duodenum (1/3 have trisomy 21 and is also associated with other congenital malformations) - Artesia or stenosis of ileum/jejunum - malrotation with volvulus (can lead to infarction of the entire midgut) - meconium ileus (in lower ileum) - meconium plug (lower intestinal obstruction) -> can also have spontaneous intestinal perforation
241
In what neonates is meconium ileus more common?
in neonates with CF
242
Causes for large bowel obstruction in neonates
- Hirschsprung Disease - absence of the myenteric nerve plexus in the rectum (may extend to the colon) - rectal atresia - absence of the anus at the normal site
243
Epidemiology of Hirschsprung disease
- More common in boys - more common in Down syndrome
244
What types of rectal atresia can you have and what are the anatomical differences?
- lesions are high or low depending on if the bowel ends above or below the lavatory ani muscle. - high lesions: fistula to the bladder or urethra in boys, Orr adjacent the vagina or to the bladder in girls.
245
what is exomphalos/omphalocele?
the abdominal contents protrude through the umbilical ring covered with a transparent sac formed by the amniotic membrane and peritoneum. It is often associated with other major congenital abnormalities.
246
What is gastoschisis?
- bowel protrudes through an opening in the anterior abdominal wall adjacent to the umbilicus, and there is no covering sac. - no association with other congenital abnormalities. - carries a (much greater than exomphalos) risk of dehydration and protein loss.
247
Management of gastroschisis
- cover abdomen with clear occlusive wrap to minimise fluid and protein loss - NG tube is passed and aspirated frequently - IV fluids Definitive treatment: - primary closure of the abdomen - large lesions: intestine is enclosed in a silastic sac sutured to the edges of the abdominal wall and contents are gradually returned into the peritoneal cavity.
248
Classical x-ray finding in duodenal artrersia?
double bubble
249
What vaccines are contraindicated in immunosuppressed patients? Give an example
live-attenuated vaccines MMR
250
Which interleukin is responsible for fevers? What cells secrete it?
IL-1 is the major endogenous pyrogen and is responsible for the generation of most fever symptoms. It is secreted by macrophages
251
What cells secrete IL-1?
macrophages
252
What is the role of IL-1?
stimulates T-cells, B-cells, neutrophils, fibroblasts and epithelial cells to grow, differentiate and synthesise specific products.
253
What does HEADSSS stand for?
H - Home E - education and employment A - activities D - drugs/drinking S - Sex S - self-harm, depression, suicide S - safety (incl. social media/online)
254
What electrolyte imbalance can be a complication of RSV bronchiolitis ?
Hyponatraemia (This can potentially lead to seizures.)
255
What % of bronchiolotis is due to RSV?
80%
256
Common causes of meningitis in neonates
Gp B Strep, E Coli, Listeria, Pneumococcus, Staph aureus.
257
Common causes of meningitis in babies over 1 month
Meningococcus, Pneumococcus and Haemophilus influenzae B (Haemophilus was previously more common until it was included in the childhood vaccination programme; it now much less commonly seen).
258
What is the most common causative organism of childhood meningitis?
Neissera meningitides 5% of survivors have neurological sequelae (not all of them) and there is a 10% mortality
259
What is the incubation period for chicken pox?
3w
260
When are patients with chicken pox infectious?
from up to 3 days before the lesions appear until when the lesions crust over.
261
Criteria for definition of chronic illness
- lasts or is expected to last at least 3 months - affects the child's age-appropriate activities - results in extensive utilisation of the healthcare system
262
What other conditions should children with T1DM be screened for?
- coeliac disease (at Dx and then annually) - thyroid disease (at Dx and then annually) - retinopathy (12+) - microalbuminuria (12+) - BP - annual foot care review (12+) - injection site: investigation of state at each visit (skin changes can lead to insulin being released a lot later, leading to highs and lows in glucose levels)
263
What can happen to blood glucose levels in patients with T1DM when they have an infection?
glucose may rise and insulin requirement may rise by 20% in both viral and bacterial illnesses. Insulin should be given even if the patient is not eating. Should also check for blood ketones; if rising -> extra insulin; If rising despite extra insulin -> hospital, risk of DKA
264
What is the risk of developing T1DM when you have a close relative (parent/sibling) with T1DM?
6%
265
What is the risk of developing T1DM if you don't have a close relative with the disease?
0.5%
266
What vitamin is folic acid?
B9
267
What proportion of children (2-15yo) in the UK are overweight or obese?
1/3
268
What are the most common nutritional deficiencies in children in the UK?
iron Vit D
269
What are the abnormalities seen in Tetralogy of Fallot
- RV hypertrophy - overriding aorta - VSD - pulmonary stenosis
270
Features of meconium ileus on AXR
bubbly appearance of intestines lack of air fluid levels
271
Features of benign murmurs in childhood
Soft Systolic (note: all diastolic murmurs are pathological) Sensitive (changes with the child's position / alongside respiration) Short (not holosystolic) Single (no additional sounds) Small (localised, non-radiating)
272
why can ibuprofen be used to help dose the ductus arteriosus?
prostaglandins keep the duct open. Ibutprofen is a prostaglandin inhibitor.
273
What medication can be used to maintain a patent ductus arteriosus?
Alprostadil (PGE1) - because prostaglandins will prevent the ductus arteriosus from closing.
274
Definition of patent ductus arteriosus
When the ductus arteriosus remains open past 4 weeks of life
275
What infection are Koplik's spots associated with?
Measles
276
What virus is commonly responsible for febrile seizures?
Roseola (HHV-6)
277
Does fever above 40 C indicate bacterial infection?
No, can also be due to viral infections.
278
What is the typical age group for simple febrile seizures?
1-5yo
279
What is the incidence of Meckel's diverticulum?
2% of people have it. Only a small proportion of children have issues with it; males are more likely to develop complications than females
280
What is the pathology in Meckel's Diverticulum?
An outpouching of the small intestine with tissue from the stomach/pancreas forms. cells from the stomach can sect acid and cause ulceration and bleeding.
281
Most common cause of haemodynamic shock in children
sepsis
282
Management of haemodynamic shock in children
1. oxygen 2. vascular access (IV, IO) - fluid boluses immediately required? ? ABx 3. blood tests and gas 4. other tests
283
What can trigger onset of T1DM?
viral infections
284
% of adolescents that self harmed in the past 12 months?
7-9%
285
What is the prevalence of ASD?
3-6/1000 Live births or 7.6/1000 worldwide prevalence
286
ASD more common in boys or girls?
boys
287
When does ASD usually present?
between 2-4 years of age when language and social skills usually expand rapidly
288
What is the commonest pathogen to cause septic arthritis in immunocompetent children?
Staphylococcus aureus
289
What is the commonest cause of septic arthritis in children with sickle cell?
Staphylococcus aureus
290
What is the commonest route of pathogen spread leading to septic arthritis?
haematological spread
291
What is another name for IgA vasculitis?
HSP
292
Does post-strep glomerulonephritis give you a rash?
no
293
What is a complication of measles infection?
- pneumonia - encephalitis - immunosuppression - subacute sclerosis pan encephalitis (can occur up to 10 years after initial measles infection)
294
Why is parvovirus B19 dangerous to pregnant women?
- can cause cross the placenta and cause severe anaemia in the fetus due to viral suppression of fetal erythropoiesis - This causes hydrops fetalis and carries a high risk of intrauterine death.
295
What is hydrocephalus?
- the result of an excess of CSF accumulating in the brain's ventricular system - puts pressure on the brain parenchyma and can have devastating neurological consequences if not recognised and treated
296
What antibodies most commonly cause rhesus disease in newborns?
Anti-rhesus D IgG antibodies transferred from mum to baby via the placenta
297
Aspirin regimen in children with Kawasaki disease
Initially 7.5–12.5 mg/kg 4x/day for 2 weeks or until afebrile Then 2–5 mg/kg once daily for 6–8 weeks if no evidence of coronary lesions after 8 weeks, discontinue treatment or seek expert advice. ref: BNFC https://bnfc.nice.org.uk/drugs/aspirin/
298
Causative agent of whooping cough
Bordetella pertussis
299
What are the 3 ossicles in the middle ear (from tympanic membrane to the oval window)
malleus, incus, stapes
300
How to distinguish between the left and right ear when looking at a picture of the tympanic membrane?
look where the short process of malleus is pointing to Pointing to right -> right ear Pointing to left -> left ear
301
Symptoms of otitis externa
- irritable child - worsening otalgia - otorrhoea - itchiness - ear fullness or hearing loss - tinnitus
302
Risk factors for otitis externa
young children f>m frequent contact with water obstruction of ear canal foreign body left in ear eczema/psoriasis immunocompromised patients ear trauma
303
otoscope findings in bacterial otitis externa
- narrow ear canal - tympanic membrane is not visible - swollen and erythematous ear - yellow/white crusted discharge
304
Otoscopy findings in fungal otitis externa
- narrow ear canal - tympanic membrane not visible - swollen and erythematous ear canal - thick white/gray discharge with spores
305
Difference in management of bacterial and fungal otitis externa
- different agents - in fungal infections treatment may be longer lasting up to 6 weeks
306
Commonest causative agents of otitis externa
upper resp tract pathogens - Pseudomonas aeruginosa (40%) - S. epidermidis - S. aureusa - anaerobes - Fungal infection (typically Aspergillus spp.)
307
Management of otitis externa
1. Education (no ear buds, dont put anything e.g. hearing aids in your ear, keep ears dry ) 2. SWAB 3. ear micro suction 4. ear drops, antibiotics, steroid or antifungals.
308
RFs for acute otitis media
- young children going to nursery - male - passive smoking - bottle feeding (decreased immune response) - craniofacial abnormalities (cleft palate, down syndrome) - large adenoids
309
commonest organisms for acute otitis media (AOM)
RSV Rhinovirus streptococcus pneumonia haemophilus influenzae
310
Why are children more predisposed to acute otitis media compared to adults?
Eustachian tube size and angulation; thinner and flat Eustachian tube, easier for infection to spread from the nose and posterior nasopharynx to the middle ear.
311
What type of tissue lines the Eustachian tube?
same tissue as nasopharynx
312
Course of acute otitis media
URTI -> symptoms a. tympanic membrane perf b. mastoiditis or c. gets better
313
Management of mastoiditis
ENT emergency Hospital admission and IV antibiotics
314
Treatment of acute otitis media
- 80 % are self limiting and resolve within 3 days if >4d consider oral abx consider IV abs if <2yo, systemically unwell or immunocompromised
315
Management of recurrent acute otitis media
- grommet insertion +/- adenoidectomy
316
What is recurrent otitis media?
at least 6 episodes in a year
317
complicactions of acute otitis media
- tympanic membrane perf - OM with effusion - intracranial complications (mastoiditis, meningitis, subdural abscess, brain abscess, extradural abscess, labyrinthitis, facial paralysis, lateral sinus thrombophlebitis)
318
causes of tympanic membrane perforation
- loud sounds - trauma - infection - foreign bodies in the ear - iatrogenic - cholesteatoma
319
symptoms of TM perforation
- sudden (unilateral) hearing loss - tinnitus - otalgia - otorrhoea
320
What is glue ear?
otitis media with effusion presence of fluid in the middle ear for >3 months
321
peak incidence age of glue ear and time of year
2-5 more common in winter
322
otitis media with effusion on otoscopy
air fluid level (bubbles may be seen) dull/opaque TM
323
tuning fork frequency in ENT and neurology tuning fork
ENT: 512 Hz Neuro: 256 Hz
324
Imaging in mastoiditis
contrast enhanced CT petrous bone +/- brain
325
What are the types of hearing tests you can do in newborns?
AOAE (automated otoacoustic emissions) - clicking sound, echo is reflected back into canal and measured by microphone; hearing loss if no echo or reduced echo; but this can also be caused by unsettled child, background noise or temporary blockage in ear -> offer ABR ABR (auditory brainstem response) - sound via mini headphones, electrodes pick up responses from the hearing nerve; best hearing test from newborns.
326
What kind of biopsy is necessary to confirm Hirschsprung disease?
Rectal Suction biopsy at least 1.5 cm above pectinate line; to demonstrate acetylcholinesterase positive nerve excess and the absence of ganglion cells.
327
Management of Hirschsprung disease
Surgical removal of the section of aganglionic colon and the healthy bowel is pulled through
328
What is the commonest place for a nosebleed?
Kiesselbach's plexus (=Little's area) it is a network of arteries in the front of the nasal cavity
329
Why is Kiesselbach's plexus the commonest plit is a network of arteries in the front of the nasal cavityace for a nosebleed?
It is where 2 big arteries anastomose it is a network of arteries in the front of the nasal cavity
330
Where and why would you apply ice during epistaxis?
back of the neck and the forehead; in order to vasoconstrict some of the main vessels supplying blood to the nose
331
symptoms of adenoidal hypertrophy
persistent mouth breathing hypo nasal speech obstructive sleep apnoea otitis media with effusion acute otitis media
332
Management of periorbital cellulitis
- IV abs (e.g. high dose ceftriaxone) - +/- surgical drainage - nasal decongestant
333
imaging in periorbital cellulitis
CT or MRI scan of the head (incl. sinuses?)
334
What are sources of infection leading to periorbital cellulitis?
- sinuses (e.g. maxillary, frontal, ethmoid cells) - trauma to local skin - dental abscess
335
What are complications of periorbital cellulitis?
- posterior spread leading to orbital cellulitis - meningitis - cavernous sinus thrombosis - abscess formation
336
symptoms and hx of periorbital cellulitis
fever swelling, tenderness and/or erythema of the eyelid/skin surrounding the eye proptosis acute eye swelling preceded by an URTI -> examine eye movements every time you see the child.
337
Symptoms of orbital cellulitis
- swelling, erythema and tenderness of the eyelid/swelling adjacent to the eye (periorbital cellulitis) - proptosis - painful and limited ocular movement - +/- reduced visual acuity
338
do foreign bodies in the ear and nose have to be removed on the same day?
In the nose, yes because there is a risk of inhalation. In the ears it depends on the object how urgently it has to be removed ranging from right away to at the next available appointment.
339
What investigations for foreign body ingestion?
AP and lateral CXR
340
Causative organisms of tonsillitis
Same as URTI - resp viruses - haemophilus influenza - pneumococcus - haemolytic strep
341
Indications for ENT referral in tonsillitis
- recurrent debilitating tonsillitis - asymmetrical tonsillitis / unilateral enlargement - sleep disordered breathing in children
342
Management of epiglottitis
hospital admission intubation and IV steroids
343
What is coarctation of the aorta?
A narrowing of the aorta. It is typically just before the ductus arteriosus.
344
What condition is coarctation of the aorta associated with?
Turner's syndrome
345
Presentation of coarctation of the aorta
- asymptomatic if mild - systolic murmur, loudest between scapulae - radio-femoral delay (radio-radial depending on site and severity) - If severe, neonates present in shock once the ductus arteriosus closes due to low perfusion pressures after the coarctation - hypertension - heart failure
346
What are the two classifications of aortic coarctation?
- classified according to whether the narrowing occurs before or after the ductus arteriosus (pre-ductal or post-ductal)
347
Management of coarctation of the aorta
- Monitoring via echocardiography and antihypertensives are used to control BP if needed - Most cases requiring intervention are corrected via angioplasty and stent insertion - Severe cases require surgery - Neonates presenting in shock due to critical coarctation require prostaglandin to keep the ductus arteriosus patent until the defect is corrected
348
Presentation of Turner's syndrome
Short stature Lymphoedema of hands and feet in neonate, may persist Spoon-shaped nails Webbed neck Widely space nipples Wide carrying angle Congenital heart defects - bicuspid aortic valve (most common), coarctation of the aorta Delayed puberty Ovarian dysgenesis causing infertility Hypothyroidism Recurrent otitis media Normal intellect
349
Incidence of Turner syndrome
1 in 2500 live births
350
Management of Turner's syndrome
- GH therapy - oestrogen replacement to allow development of secondary sexual characteristics
351
How do you manage croup? incl drug dose
single dose of oral dexamethasone 0.15 mg/kg
352
What pathogen is most commonly responsible for bronchiolitis obliterans?
Adenovirus
353
What is bronchiolitis obliteratans?
a complication of bronchiolitis usually caused by adenovirus scarring to the airways resulting in permanent narrowing
354
What drugs cause QT-interval prolongation?
- macrolides (e.g. erythromycin, clarithromycin, azithromycin) - quinolones (ciprofloxacin) - tricyclines - antipsychotics - ondansetron
355
What is the commonest cause of nephrotic syndrome in children?
Minimal change disease
356
Management of Impetigo
Hydrogen peroxide 1% cream topical fusidic acid
357
Indications for a head CT following head trauma in a child
Suspicion of non-accidental injury Post-traumatic seizure but no history of epilepsy. On initial emergency department assessment, GCS less than 14, or for children under 1 year GCS (paediatric) less than 15. At 2 hours after the injury, GCS less than 15. Suspected open or depressed skull fracture or tense fontanelle. Any sign of basal skull fracture (haemotympanum, 'panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign). Focal neurological deficit. For children under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head. A provisional written radiology report should be made available within 1 hour of the scan being performed. https://www.nice.org.uk/guidance/cg176/chapter/Recommendations#investigating-clinically-important-brain-injuries
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What is the most common form of congential heart defect in infants of diabetic mothers?
transposition of the great vessels
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VSD on auscultation
pan systolic murmur
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What is the most common congenital heart defect?
VSD 30-60% of all congenital heart defects
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Fluid prescription in dehydrated children over 24h
maintenance fluids + weight (kg) x 10 x %dehydration (e.g. 25 x 10 x 5 for a 25kg child with 5% fluid loss = 1600 + 1250 = 2850)
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Benzylpenicillin doses for suspected meningitis
1-11 mo: 300 mg IM 1-9 yo: 600 mg IM 10-17 yo: 1.2 g IM BNFC: IM or IV
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What ABx are used for Scarlet fever?
Penicillin V
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When is hand preference worrying?
If before 18 months of age should not have hand preference before 2 yo could indicate hemiplagia or cerebral palsy
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Define intussusception
invagination of proximal bowel into a distal segment
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Commonest cause of intestinal obstruction in infants after the neonatal period?
intussusception
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at what age does intussusception occur
may occur at any age peak is 3 months to 2 years
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complications of intussusception
- most complicated: stretching and constriction of the mesentery resulting in venous obstruction, causing engorgement and bleeding from the bowel mucosa, fluid loss and subsequently bowel perforation, peritonitis and gut necrosis. - hypovolaemic shock (fluid may be pooling in the intestine)
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Management of Impetigo
- hygiene measures (washign hands after touching lesions) - school exclusion after the lesions have crusted over - first line: topicaread diseases: orgal flucloxacillin in0 oral clarithromycin