Paediatrics Flashcards

1
Q

-What is pneumonia?

A

An infection of the lung tissue. It causes inflammation of the lung tissue and sputum filling the airways and alveoli

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2
Q

What is shown on an x-ray of pneumonia?

A
  • Consolidation
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3
Q

What is the presentation of pneumonia?

A
  • Cough
  • High fever
  • Tachypnoea
  • Tachycardia
  • Increased work of breathing
  • Lethargy
  • Delirium
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4
Q

What are the signs of pneumonia?

A

Hypoxia (low oxygen)
Hypotension (shock)
Fever
Confusion

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5
Q

What are the characteristic chest signs of pneumonia?

A
  • Bronchial breath sounds
  • Focal coarse crackles
  • Dullness to percussion
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6
Q

What is the most common cause of pneumonia?

A

Strep Pneumonia

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7
Q

What are the common types of pneumonia in unvaccinated children?

A
  • Group B strep contracted during birth
  • Haemophilus influenza
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8
Q

What bacteria produces a chest x-ray finding of pneumatoceles?

A

S.aureus

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9
Q

What is an atypical pneumonia in children?

A

Mycoplasma pneumonia has extra pulmonary manifestations such as erythema multiforme

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10
Q

What are the viral causes of pneumonia?

A
  • Respiratory syncytial virus (RSV) is the most common
  • Parainfluenza
  • Influenza virus
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11
Q

What is the management for pneumonia?

A
  • Amoxicillin used first line- adding macrolide (clarithromycin will cover atypical)
  • Macrolide also be used in Pen allergy
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12
Q

What needs to be tested in children with recurrent LRTI?

A
  • Full blood count rule out leukaemia
  • Chest x-ray to rule out structural abnormality
  • Serum immunoglobulins
  • Test response to previous vaccines- some patients can’t convert IgM to IgG
  • Sweat test for CF
  • HIV test
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13
Q

What is croup?

A
  • Typically affects children from 6months to 2 years.
  • It is an URTI and causes oedema in the larynx
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14
Q

What is the most common cause of Croup?

A

Parainfluenza

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15
Q

What are some other causes of croup?

A

Influenza
Adenovirus
Respiratory Syncytial Virus (RSV)

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16
Q

What type of croup is associated with a high mortality?

A

Diphtheria- leads to epiglottitis which has a high mortality rate

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17
Q

What is the presentation of croup?

A
  • Increased work of breathing
  • Barking cough
  • Hoarse voice
  • Stridor
  • Low grade fever
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18
Q

What is the management for Croup?

A
  • Oral dexamethasone of 150mcg/kg
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19
Q

What is the stepwise treatment of croup?

A
  • Oral dexamethasone
  • Oxygen
  • Nebulised budesonide
  • Nebulised adrenalin
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20
Q

What is the presentation of an acute exacerbation of asthma?

A
  • Worsening shortness of breath
  • Signs of respiratory distress
  • Fast respiratory rate
  • Expiratory wheeze

A silent chest is an ominous sign as the child can’t get enough air to even make a wheeze

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21
Q

What is a moderate severity asthma attack?

A

Peak flow > 50% predicted
Normal speech

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22
Q

What is a severe asthma attack?

A

Peak flow <50% predicted
Sats <92%
Unable to complete sentences in one breath
Signs of respiratory distress

Respiratory rate:
> 40 in 1-5 years
> 30 in > 5 years

Heart rate
> 140 in 1-5 years
> 125 in > 5 years

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23
Q

What is a life-threatening asthma attack?

A

Peak flow < 33% predicted

Exhaustion and poor respiratory effort

Hypotension

Silent chest

Cyanosis

Altered consciousness / confusion

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24
Q

What is the management of an acute asthma attack?

A

Salbutamol inhalers via a spacer device: starting with 10 puffs every 2 hours

Nebulisers with salbutamol / ipratropium bromide

Oral prednisone (e.g. 1mg per kg of body weight once a day for 3 days)

IV hydrocortisone

IV magnesium sulphate

IV salbutamol- make sure to check potassium levels

IV aminophylline

If not under control then may need intubation

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25
What are the key presentations of asthma?
- Episodic symptoms - Diurnal variability - Typical triggers - A history of other atopic conditions such as eczema, hay fever and food allergies - Family history of asthma or atopy - Bilateral widespread “polyphonic” wheeze heard by a healthcare professional - Symptoms improve with bronchodilators
26
What presentation suggests a diagnosis other than asthma?
- Wheeze only related to coughs or colds - Isolated or productive cough - Normal investigations - No response - Unilateral suggests a focal lesion or foreign body
27
What is is used to make a diagnosis of asthma?
Spirometry with reversibility testing (in children aged over 5 years) Direct bronchial challenge test with histamine or methacholine Fractional exhaled nitric oxide (FeNO) Peak flow variability measured by keeping a diary of peak flow measurements several times a day for 2 to 4 weeks
28
What is the medical treatment in chidren under the age of 5 with asthma?
- SABA as required - Steroid or leukotriene antagonist
29
What is the treatment for asthma aged 5-12?
Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required Add a regular low dose corticosteroid inhaler Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response. Titrate up the corticosteroid inhaler to a medium dose. Consider adding: Oral leukotriene receptor antagonist (e.g. montelukast) Oral theophylline Increase the dose of the inhaled corticosteroid to a high dose. Referral to a specialist. They may require daily oral steroids.
30
Start a short-acting beta 2 agonist inhaler (e.g. salbutamol) as required Add a regular low dose corticosteroid inhaler Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response. Titrate up the corticosteroid inhaler to a medium dose. Consider a trial of an oral leukotriene receptor antagonist (i.e. montelukast), oral theophylline or an inhaled LAMA (i.e. tiotropium). Titrate the inhaled corticosteroid up to a high dose. Combine additional treatments from step 4, including the option of an oral beta 2 agonist (i.e. oral salbutamol). Refer to specialist. Add oral steroids at the lowest dose possible to achieve good control under specialist guidance.
31
What is the problem with giving a child a steroid inhaler?
- Some parents are worried that they can slow growth of up to (1cm) when used long term - This effect is dose-dependant meaning it is less of a problem with smaller doses
32
How often do spacers need to be cleaned?
Once a month and make sure they are air dried as static can prevent medication inhalation
33
What is a viral induced wheeze?
Small children under the age of 3 have small airways. So when the encounter a virus they develop inflammation and oedema
34
Why are children's airways more affected with inflammation and oedema?
Swelling and constriction of the airway caused by a virus has little noticeable effect on the larger airways of an older child or adult, however due to the small diameter of a child’s airway, the slight narrowing leads to a proportionally larger restriction in airflow. This is described by Poiseuille’s law, which states that flow rate is proportional to the radius of the tube to the power of four. Therefore, halving the diameter of the tube decreases flow rate by 16 fold.
35
What is a major risk factor for a viral induced wheeze?
Family history
36
What is the difference between asthma and a viral induced wheeze?
Presenting before 3 years of age No atopic history Only occurs during viral infections
37
What is the presentation of a viral induced wheeze?
Evidence of a viral illness (fever, cough and coryzal symptoms) for 1-2 days preceding the onset of: Shortness of breath Signs of respiratory distress **Expiratory wheeze throughout the chest**
38
What causes a focal wheeze?
Not asthma or viral If you hear a focal wheeze be very cautious and investigate further for a focal airway obstruction such as an inhaled foreign body or tumour.
39
What is Bronchiolitis?
Describes inflammation and infection in the bronchioles.
40
What is the most common cause of Bronchiolitis?
RSV
41
What ages is Bronchiolitis most comon?
- Most common in under a year- particularly in under 6 months - Can occur in older children particularly in premature babies with chronic lung disease
42
What is the presentation of Bronchiolitis?
- Coryzal symptoms - Signs of resp distress - Dyspnoea - Tachypnoea - Poor feeding - Mild fever - Apnoea's are episodes where the child stops breathing - Wheeze and crackles on auscultation
43
What are the signs of respiratory distress?
- Raised Resp rate - Use of accessory muscles for breathing - Intercostal and subcostal recessions - Nasal flaring - Head bobbing - Tracheal tugging- downward displacement during inspiration - Cyanosis - Abnormal airway noises
44
What are some abnormal airway noises?
Wheezing is a whistling sound caused by narrowed airways, typically heard during expiration Grunting is caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure Stridor is a high pitched inspiratory noise caused by obstruction of the upper airway, for example in croup
45
What are reasons for admission with bronchiolitis?
- Aged under 3 months or any pre-existing condition - 50-75% reduction in milk intake - Clinical dehydration - Resp rate over 70 - Oxygen sats below 92% - Head bobbing - Apnoea's
46
What is the management for bronchiolitis?
Ensuring adequate intake. This could be orally, via NG tube or IV fluids depending on the severity. It is important to avoid overfeeding as a full stomach will restrict breathing. Start with small frequent feeds and gradually increase them as tolerated. Saline nasal drops and nasal suctioning can help clear nasal secretions, particularly prior to feeding Supplementary oxygen if the oxygen saturations remain below 92% Ventilatory support if required
47
What is useful for assessing ventilation in respiratory distress?
Capillary blood gases Rising pCO2- Shows the airways have collapsed and can't clear CO2 Falling pH- shows the CO2 is building up and not able to buffer the acidosis
48
What is given to high risk babies for bronchiolitis?
**Palivizumab** is given as monthly injection to prevent it
49
What is cystic fibrosis?
An inherited autosomal recessive multi-system disease affecting the mucus glands
50
A mutation on what gene causes CF?
cystic fibrosis transmembrane conductance regulatory gene on **chromosome 7**
51
How does a mutation to the CFTR gene cause disease?
- The CFTR protein gets misfolded and can't migrate from the RER to the cell membrane - The CFTR is a channel protein that pumps chloride ions into various secretions helping to thin them out meaning secretions are left overly thick
52
How does CF cause respiratory problems?
- Results in dry airways and impaired mucociliary clearance - The low volume thick airway secretions result in reduced airway clearance increasing chances of infection and this chronic inflammation can lead to bronchiectasis
53
What are the GI problems associated with CF?
- Thickened secretions within small and large bowel can make it difficult to pass stools resulting in bowel obstruction
54
What are the pancreatic problems associated with CF?
Thick pancreatic and bile secretions can block the pancreatic ducts resulting in a lack of digestive enzymes this can also result in pancreatitis and diabetes
55
What are the liver problems associated with CF?
Thickened biliary secretions may block the bile ducts resulting in liver fibrosis and cirrhosis
56
What are some other problems associated with CF?
- Can result in pulmonary hypertension leading to right sided heart failure - In males there is bilateral absence of vas deferens so it means there is male infertility
57
What if often the first sign of CF in a baby?
**meconium ileus**
58
What is meconium ileus?
- In babies the first stool passed is called the meconium and it is black and sticky and should be passed within 48 hours - In babies with CF the meconium does not pass as it is too sticky so it causes bowel obstruction occurs in 20% of babies with CF
59
What are some signs of CF?
- Low weight - Nasal polyps - Finger clubbing - Crackles and wheezes - Abdominal distension
60
What are the symptoms of CF?
- Chronic cough - Thick sputum production - Recurrent respiratory infections - Loose, greasy stools (steatorrhea) due to a lack of fat digesting lipase enzymes - Abdominal pain and bloating - Poor weight and height gain (failure to thrive) -Parents may report the child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat
61
When is CF most often diagnosed?
- It is found during the **heel-prick/Guthrie test** which screens for CF in babies by looking for serum immunoreactivity trypsinogen
62
What is the gold standard test for CF?
The sweat test
63
What is the sweat test?
test for CF - **Pilocarpine** is applied to the skin and electrodes are placed either side of the patch with small current to cause skin to sweat - The sweat is absorbed and sent to lab for testing a diagnostic test of chloride concentration above **60 mmol/l is diagnostic
64
What are common microbial colonisers in CF?
- Staphylococcus aureus- patients take long term prophylactic flucloxacillin - Pseudomonas aeruginosa- can be harder to treat and worsen the prognosis
65
What is the management for the respiratory symptoms of CF?
- Chest physiotherapy at least twice a day to remove mucus - Exercise - Salbutamol - Nebulised DNase (dornase alfa wolf) an enzyme that breaks down DNA material in respiratory secretions - Nebulised hypertonic saline
66
What is the treatment for the GI symptoms of CF?
- CREON tablets helps to digest fats in patients with pancreatic insufficiency (missing lipase) - High calorie diet to make up for malabsorption and calories needed for respiratory effort
67
What is the prognosis for CF?
. Life expectancy is improving and currently the cystic fibrosis trust gives a median life expectancy of 47 years. - 90% of patients with CF develop pancreatic insufficiency - 50% of adults with CF develop cystic fibrosis-related diabetes and require treatment with insulin - 30% of adults with CF develop liver disease Most males are infertile due to absent vas deferens
68
what is the new miracle cure for CF?
Kaftrio, described by patient groups as a ‘revolutionary drug’, is a triple combination treatment combining three drugs which perform different functions – ivacaftor, tezacaftor and elexacaftor – and tackles the underlying causes of the disease, by helping the lungs work effectively.
69
What is epiglottitis?
Inflammation and swelling of the epiglottis caused by infection. The epiglottis can swell to the point of completely obscuring the airway within hours of symptoms developing
70
What is the main cause of epiglottitis?
Haemophilus influenza B
71
What is the presentation of a potential epiglottitis?
- Sore throat and stridor - **Drooling** - **Tripod position** - High fever - Muffled voice - Septic and unwell appearance
72
What are the investigations for epiglottitis?
- If patient is unwell and it is suspected then do not perform investigations - Lateral x-ray of the neck will show **thumb sign**
73
What is the management for epiglottitis?
- Consult an anaesthetist and make sure that the airway is secure - Once airway is secure give IV ceftriaxone and dexamethasone
74
What is a common complication of epiglottitis?
An epiglottic abscess
75
76
What is otitis media?
- Infection of the middle ear which is the space that sits between the tympanic membrane and the inner ear - This is where the nerves are found
77
What causes otitis media?
- Bacteria enter from the back of the thorat through the eustachian tube - A Viral URTI often precedes otitis media
78
What is the most common cause of otitis media?
Strep pneumoniae
79
What is the presentation of otitis media?
- **Ear pain** - Reduced hearing - Feeling unwell - Signs of URTI
80
Examination otitis media?
- Otoscope tympanic membrane will look bulging red and perforation will show discharge and hole in tympanic membrane
81
Management otitis media?
Most otitis media cases will resolve without antibiotics within around three days, sometimes up to a week. Antibiotics make little difference to symptoms or complications. Amoxicillin for 5-7 days first-line Clarithromycin (in pencillin allergy) Erythromycin (in pregnant women allergic to penicillin)
82
When should antibiotics be prescribed in otitis media?
- Symptoms lasting longer than 4 days - Systemically unwell - Immunocompromised - Younger than 2 years with bilateral - Perforation/discharge in canal
83
What is Glue ear?
It is known as otitis media with effusion. The middle ear becomes full of fluid and causes ;loss of hearing in that ear
84
What is the tube that connects the middle ear to the throat called?
The Eustachian tube connects the middle ear to the back of the throat. It helps drain secretions from the middle ear. When it becomes blocked, this causes middle ear secretions (fluid) to build up in the middle ear space.
85
What is the main symptom of glue ear?
Reduction in hearing in that ear.
86
What will otoscopy show in glue ear?
A dull tympanic membrane with air bubbles or a visible fluid level
87
What is the management for glue ear?
Referral for audiometry to help establish the diagnosis and extent of hearing loss. Glue ear is usually treated conservatively, and resolves without treatment within 3 months. Children with co-morbidities affecting the structure of the ear, such as Down’s syndrome or cleft palate may require hearing aids or grommets.
88
What are grommets?
Tiny tubes inserted into the tympanic membrane by an ENT surgeon. This allows fluid to drain from the middle ear Grommets usually fall out within a year and don't need to be reinserted
89
What are some congenital causes of deafness?
- Maternal rubella or CMV - Genetic deafness - Associated syndromes e.g. down's syndrome
90
What are some perinatal causes of deafness?
- Prematurity - Hypoxia during or after birth
91
What are some causes of birth after birth?
- Jaundice - Meningitis and encephalitis - Otitis media and glue ear - Chemotherapy
92
How is a child tested for deafness?
- Newborn hearing screening assessment tests hearing in all neonates
93
What are signs that a child may have hearing difficulties?
Ignoring calls or sounds Frustration or bad behaviour Poor speech and language development Poor school performance
94
What are audiograms?
- Charts that document the volume at which patients can hear different tones. The frequency in hertz is plotted on x-axis and decibels on Y axis - It is recorded as the minimum volume required to hear each frequency, the worse the hearing the lower down on the chart - Both air and bone conduction are tested separately
95
What are the symbols used to mark on the chart?
X – Left sided air conduction ] – Left sided bone conduction O – Right sided air conduction [ – Right sided bone conduction
96
What will patients with sensorineural hearing loss show on the chart?
**Both air and bone conduction** readings will be more than **20 dB, plotted below the 20 dB** line on the chart.
97
What will patients with conductive hearing loss show on the chart?
In patients with conductive hearing loss, bone conduction readings will be normal (between 0 and 20 dB), however air conduction readings will be greater than 20 dB, plotted below the 20 dB line on the chart. In conductive hearing loss, sound can travel through bones but is not conducted through air due to pathology along the route into the ear.
98
What is mixed hearing loss?
In patients with mixed hearing loss, both air and bone conduction readings will be more than 20 dB, however there will be a difference of more than 15 dB between the two (bone conduction > air conduction).
99
Give 3 causes of conductive hearing loss.
1. Glue ear. 2. Ear wax. 3. Otitis media. 4. Perforated ear drum.
100
Give 3 risk factors for sensori-neural hearing loss?
ongenital infection, eg rubella, CMV Meningitis Genetics Consanguinity. Usher Syndrome hypoxic-ischemic injury to the brainstem, hemorrhage into the inner ear, toxic effects of bilirubin or aminoglycoside,
101
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.
102
How would you manage mixed hearing loss?
You would address the conductive problem first and then offer a hearing aid.
103
What is seen in peri orbital cellulitis?
- In periorbital cellulitis there is fever with erythema, tenderness and oedema of the eyelid or other skin adjacent to the eye
104
How should you treat periorbital cellulitis?
- It should be treated quickly with intravenous antibiotics such as **high-dose ceftriaxone** to prevent posterior spread which can cause orbital cellulitis
105
What is a squint?
- It refers to the misalignment of the eyes **strabismus** - In childhood the brain will cope with this misalignment by reducing the signal from the less dominant eye hence a squint - If this is not treated the lazy eye becomes disconnected from the brain and the problem becomes worse this is called **amblyopia**
106
What are the two types of squint?
Concomitant (non paralytic) squints are due to differences in the control of the extra ocular muscles. The severity of the squint can vary. Paralytic squints are rare. They are due to paralysis in one or more of the extra ocular muscles.
107
Outline the two tests used to detect squints.
orneal light reflex test A Light in shone from a distance at both corneas simultaneously. If the light reflection does not appear in the same position in the two pupils, a squint is present Cover test The child is encouraged to look at a toy/light. If the fixing **(dominant)** eye is covered, the *squinting eye will move to take up fixation.** Cover test should be performed at different distances
108
name some causes of paralytic squints
Infections: Infections like meningitis or encephalitis can damage the nerves or areas of the brain responsible for controlling eye movement. Birth Trauma: During delivery, if there's trauma or injury to the baby's head or the nerves controlling eye movement, it can lead to a squint. Neurological Disorders: Cerebal Palsy and other conidtiosn that hinder muscle control and coordination can also result in paralytic squints. Tumors or Growth: Any abnormal growths or tumors in or around the eye, brain, or nerves controlling eye movement can put pressure on these structures, causing paralysis or weakness. Accidents or Injuries:
109
What is a ventral septal defect?
- A congenital hole in the septum between the ventricles - They can occur in isolation but are often due to underlying genetic condition such as Down's or Turner's syndrome
110
How does blood flow in a ventral septal defect and what is the presentation of it?
- Due to increased pressure in the left compared to the right the blood will flow from right to left through the hole - As blood will still flow around the body they will remain acyanotic - However this will cause a right side overload and lead to heart failure and increased low into the pulmonary vessels leading to **pulmonary hypertension**
111
What is the presentation of a VSD?
- Poor feeding - Dyspnoea - Tachypnoea - Failure to thrive
112
What murmur does a VSD present with?
- Pan systolic murmur
113
What is the treatment for a VSD?
- Surgery if big hole - Increased risk of IE so give antibiotic prophylaxis if surgery is required
114
What causes a ASD?
During the development of the fetus the left and right atria are connected. Two walls grow downwards from the top of the heart, then fuse together with the endocardial cushion in the middle of the heart to separate the atria. **These two walls are called the septum primum and septum secondum** Defects in these two walls lead to atrial septal defects, a hole connecting the left and right atria. There is a small hole in the septum secondum called the foramen ovale. The foramen ovale normally closes at birth.
115
What are the different types of ASD?
Ostium secondum, where the septum secondum fails to fully close, leaving a hole in the wall. Patent foramen ovale, where the foramen ovale fails to close (although this not strictly classified as an ASD). Ostium primum, where the septum primum fails to fully close, leaving a hole in the wall. This tends to lead to atrioventricular valve defects making it an atrioventricular septal defect.
116
What are some complications of a ASD?
- Stroke instead of PE - AF - Pulmonary hypertension and HF - Eisenmenger syndrome - Can cause arrhythmias in early childhood
117
What is the presentation of a ASD?
- Mid systolic cresendo-descendo murmur - Also a link to migraine with aura and patent foramen ovale
118
What is Eisenmenger syndrome?
Eisenmenger syndrome occurs when blood flows from the right side of the heart to the left across a structural heart lesion, bypassing the lungs. There are three underlying lesions that can result in Eisenmenger syndrome: Atrial septal defect Ventricular septal defect Patent ductus arteriosus
119
What causes EMS?
- When pulmonary pressure is greater than the systemic pressure blood begins to flow from the right side of the heart to the left - This causes deoxygenated blood to bypass the lungs and enter the body. This causes **cyanosis**
120
What happens as a consequence of the cyanosis in EMS?
- More RBCs produced leading to polycythaemia which causes plethoric appearance and increases chances of blood clots
121
What are some findings related to right left shunt?
Cyanosis Clubbing Dyspnoea Plethoric complexion (a red complexion related to polycythaemia)
122
What is a risk factor for EMS?
Pregnancy can make the problem worse
123
What is the prognosis of EMS?
Once the syndrome has occurred it is irreversible so should be treated before it occurs
124
What is a PDA?
The ductus arteriosus normally stops functioning within 1-3 days of birth, and closes completely within the first 2-3 weeks of life. When it fails to close, this is called a “patent ductus arteriosus” (PDA). The reasons why it fails to close are unclear, but it may be genetic or related to maternal infections such as rubella. Prematurity is a key risk factor.
125
What is the murmur in PDA?
a continuous crescendo-decrescendo “machinery” murmur Have a bounding pulse
126
What are the main causes of heart failure in neonates?
Hypoplastic left heart syndrome -*left side of the heart severely underdeveloped* * Critical aortic valve stenosis * Severe coarctation of the aorta * Interruption of the aortic arch - transposition of the great arteries - **Think - all lead to obstructed systemic circulation, so causes heart failure straight away**
127
What is the main causes of heart failure in infants? (1st year of life)
- Large PDA, VSD or ASD - Anything that causes mixes of circulation
128
What is the main causes of heart failure in older children/adolescents?
More due to acquired conditions: Rheumatic fever, cardiomyopathies, Eisenmenger syndrome
129
What are some symptoms of heart failure in children?
Shortness of breath – Inability to gain weight/poor growth – Predisposition to recurrent chest infections – Hepatomegaly – Signs of specific cause –> e.g., murmur, cardiomegaly, cyanosis
130
What is some general management for heart failure in children?
* Bed rest and nurse in semi-upright position: infants in chair/seat. * Supplemental oxygen **(not in left to right shunt).** * Diet: sufficient calorie intake. * Diuretics. * Angiotensin converting enzyme inhibitors
131
name 3 cyanatoic congenital heart diseases. (Right to left shunts)
Tetralogy of Fallot Transposition of the great arteries Eisenmenger
132
What are the 4 pathologies of tetralogy of Fallot?
- VSD - Overriding aorta - Pulmonary valve stenosis - Right ventricular hypertrophy
133
What are the risk factors for TOF?
- Rubella - Increased age of mother - Alcohol consumption in pregnancy - Diabetic mother
134
What is the presentation of TOF?
Most cases are picked up before the child is born during the antenatal scans. Additionally, an ejection systolic murmur caused by the pulmonary stenosis may be heard on the newborn baby check. Severe cases will present with heart failure before one year of age. In milder cases, they can present as older children once they start to develop signs and symptoms of heart failure.
135
What are the signs and symptoms of TOF?
- Cyanosis - Clubbing - Poor feeding - Poor weight gain - Ejection systolic murmur - Tet spells
136
what is a Tet spell?
- When right to left shunt is increased due to an decrease in the systemic resistance or increase in pulmonary vascular resistance - Lots of exercise produces Co2 which reduces vascular résistance - These episodes may be precipitated by waking, physical exertion or crying. The child will become irritable, cyanotic and short of breath. Severe spells can lead to reduced consciousness, seizures and potentially death.
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What is the treatment for a Tet spell?
- Position child with knees to their chest which will increase systemic vascular resistance - Beta blockers can relax the right ventricle and improve flow to the pulmonary vessels. IV fluids can increase pre-load, increasing the volume of blood flowing to the pulmonary vessels. Morphine can decrease respiratory drive, resulting in more effective breathing. Sodium bicarbonate can buffer any metabolic acidosis that occurs. Phenylephrine infusion can increase systemic vascular resistance.
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What is CoA?
Coarctation of the aorta is a congenital condition where there is narrowing of the aortic arch, usually around the ductus arteriosus. The severity of the coarctation (or narrowing) can vary from mild to severe. It is often associated with an underlying genetic condition, particularly Turners syndrome. Narrowing of the aorta reduces the pressure of blood flowing to the arteries that are distal to the narrowing. It increases the pressure in areas proximal to the narrowing, such as the heart and the first three branches of the aorta.
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What is the presentation of CoA?
*Weak femoral pulses is often the sign** - Poor feeding - Grey and floppy baby - Tachypnoea Signs over time: - Left ventricular heave - Underdevelopment of left arm - Underdevelopment of legs
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What is the management of CoA?
- Give **prostaglandin E** to keep DA open otherwise this worsens symptoms - Fix with surgery when ready
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What is Transposition of the Great Arteries?
- When the attachments of the aorta and pulmonary trunk are swapped
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What is the presentation of Transposition of the Great Arteries?
- Will often be picked up on scan - will develop respiratory distress, tachycardia, poor feeding, poor weight gain and sweating.
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What is the management Transposition of the Great Arteries?
Where there is a ventricular septal defect, this will allow some mixing of blood between the two systems and provide some time for definitive treatment. A prostaglandin infusion can be used to maintain the ductus arteriosus. This allow blood from the aorta to flow to the pulmonary arteries for oxygenation. Balloon septostomy involves inserting a catheter into the foramen ovale via the umbilicus, and inflating a balloon to create a large atrial septal defect. This allows blood returning from the lungs (on the left side) to flow to the right side of the heart and out through the aorta to the body. Open heart surgery is the definitive management. A cardiopulmonary bypass machine is used to perform an “arterial switch” procedure within a few days of birth. If present, a VSD or ASD can be corrected at the same time.
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What is rheumatic fever?
- An autoimmune condition triggered by streptococcus bacteria causes antibodies to target areas of the boddy
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What type of hypersensitivity reaction is RF?
Type II
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What bacteria most commonly causes rheumatic fever?
group A beta-haemolytic streptococcal, typically streptococcus pyogenes causing tonsillitis
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What is the presentation of RF?
Symptoms 2-4 weeks after sore throat such as: - Fever - Joint pain - Rash - SOB - Chorea - Nodules
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What are the effects of RF on different body systems?
- Joint involvement: migratory arthritis affecting the large joints, with hot, swollen, painful joints. -Inflammation throughout the heart, with pericarditis, myocarditis and endocarditis, leads to: Tachycardia or bradycardia Murmurs from valvular heart disease, typically mitral valve disease Pericardial rub on auscultation Heart failure - Subcutaneous nodules and Erythema marginatum rash - Chorea if the nervous system
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What is used to diagnose RF?
The jones criteria
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What is the Jones criteria?
Major J- Joint arthritis O- organ inflammation N- Nodules E – Erythema marginatum rash S – Sydenham chorea Minor F- Fever E- ECG Changes (prolonged PR interval) without carditis A- Arthralgia without arthritis R- Raised inflammatory markers (CRP and ESR) Two major criteria OR One major criteria plus two minor criteria
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What is the management of RF?
Pen V for 10 days
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What are some complications of RF?
Recurrence of rheumatic fever Valvular heart disease, most notably mitral stenosis Chronic heart failure
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What causes GORD in a baby?
There is immaturity of the lower oesophageal sphincter. It is normal to have reflux and fine if baby keeps gaining weight 90% resolves within a year of birth
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What are some signs of problematic reflux?
Chronic cough Hoarse cry Distress, crying or unsettled after feeding Reluctance to feed Pneumonia Poor weight gain
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What are some red flags that may make you reconsider GORD?
- Not keeping down any feed - Projectile or forceful vomiting - Bile stained - Haematemesis - Abdominal distension - Reduced consciousness, bulging fontanelle or neurological signs (meningitis or raised intracranial pressure) - Respiratory symptoms (aspiration and infection) - Blood in the stools (gastroenteritis or cows milk protein allergy) - Signs of infection (pneumonia, UTI, tonsillitis, otitis or meningitis) - Rash, angioedema and other signs of allergy (cows milk protein allergy) - Apnoeas are a concerning feature and may indicate serious underlying pathology and need urgent assessment
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What is Sandifer's syndrome?
- Abnormal movements associated with GORD Torticollis: forceful contraction of the neck muscles causing twisting of the neck Dystonia: abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
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What is the treatment for GORD?
In simple cases some explanation, reassurance and practical advice is all that is needed. Advise: Small, frequent meals Burping regularly to help milk settle Not over-feeding Keep the baby upright after feeding (i.e. not lying flat) More problematic cases can justify treatment with Gaviscon mixed with feeds Thickened milk or formula (specific anti-reflux formulas are available) Proton pump inhibitors (e.g., omeprazole) where other methods are inadequate
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What is pyloric stenosis?
- the pyloric sphincter is a ring of smooth muscle that forms between the stomach and duodenum. - Hypertrophy and therefore narrowing of this canal prevents the passage of food - Eventually due to increased peristalsis of stomach it will cause projectile vomiting from oesophagus as food can't reach duodenum
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What are the key presentations of pyloric stenosis?
- Projectile vomiting (failure to thrive in early weeks of life) - Firm round mass like a large olive in upper abdomen - Hypochloric on blood gas (metabolic alkalosis) due to vomiting of HCL
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How is pyloric stenosis diagnosed and managed?
- Abdominal ultrasound for diagnosis - a laparoscopic pyloromyotomy (known as “Ramstedt’s operation“).
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What is Gastroenteritis?
- Gastritis is inflammation of the stomach and presents with nausea and vomiting - Enteritis is inflammation of the intestines and presents with diarrhoea Gastroenteritis is inflammation all the way from the stomach to the intestines and presents with nausea, vomiting and diarrhoea.
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What are some differentials for diarrhoea?
Stools from normal babies can vary from loose stools several times a day to one stool per week. Steatorrhoea means greasy stools with excessive fat content. This suggests a problem with digesting fats, such as pancreatic insufficiency (think about cystic fibrosis). Other conditions are: - Infection - IBD - Lactose - Coeliac - CF - Toddler's diarrhoea - IBS - Medications
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What are the viral causes of Gastroenteritis?
- Rotavirus - Norovirus - Adenovirus
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What are some signs of severe dehydration and shock seen in gastroenteriris?
gns of dehydration: – Reduced urine output, lethargic, sunken eyes – However, they are haemodynamically stable (normal BP, normal capillary refill time, warm extremities) Signs of shock: – Reduced consciousness, pale skin, appears unwell – They will start to get haemodynamically compromised –> low BP, high HR, long CRT, cold extremities
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What is the treatment for gastroenteritis in children?
- Children need to stay off school for 48 hours after symptoms have resolved - Main thing is keeping the child hydrated if they can tolerate fluid then they are okay to carry on and can be discharged
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What are the antidiarrheal that can be used in gastroenteritis?
- Loperamide but **don't give antiemetic metoclopramide or others** - Also don't give medication in E.coli or shigella infections where there is bloody diarrhoea or high fever
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What are some complications of gastroenteritis?
Lactose intolerance Irritable bowel syndrome Reactive arthritis Guillain–Barré syndrome
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What are the features suggesting constipation?
- Less than 3 stools per weeks - Hard stools (rabbit dropping stools) - Abdo pain - Abnormal posture (retentive posture) - Overflow soiling - Loss of sensation of the need to open bowels
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What is encopresis?
- Only considered pathological after age of 4, it is where the rectum becomes stretched and losses sensation - Large stools remain and only loose stools can bypass and this causes soiling
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What is a concern of chronic constipation in children?
Often patients develop a habit of not opening their bowels when they need to and ignoring the sensation of a full rectum. Over time they loose the sensation of needing to open their bowels, and they open their bowels even less frequently.
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What are some red flags of constipation in children?
Not passing meconium within 48 hours of birth (cystic fibrosis or Hirschsprung’s disease) Neurological signs or symptoms, particularly in the lower limbs (cerebral palsy or spinal cord lesion) Vomiting (intestinal obstruction or Hirschsprung’s disease) Ribbon stool (anal stenosis) Abnormal anus (anal stenosis, inflammatory bowel disease or sexual abuse) Abnormal lower back or buttocks (spina bifida, spinal cord lesion or sacral agenesis) Failure to thrive (coeliac disease, hypothyroidism or safeguarding) Acute severe abdominal pain and bloating (obstruction or intussusception)
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What is the management of constipation?
- High fibre diet and good hydration - Start laxatives (movicol is first-line) - Faecal impaction may require a **impaction regimen** - Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts. Laxatives should be continued long term and slowly weaned off as the child develops a normal, regular bowel habit.
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What are some key differentials for appendicitis?
- Ectopic pregnancy - Ovarian cysts - Meckel's diverticulum - Mesenteric adenitis - Appendix mass
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What is an umbilical hernia?
The herniation of the bowel content through the umbilicus. It is a common finding in neonates and usually asymptomatic
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What are the two types of inguinal hernias?
**Direct**: the hernia protrudes through the posterior wall of the inguinal canal (20%) **Indirect**: hernia protrudes through the deep inguinal ring and into the inguinal canal
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What are the features of an inguinal hernia?
- Groin swelling - Nausea, vomiting, constipation, abdominal pain/discomfort - A scrotal mass you can't get above - Is reducible when lying flat - Does not trans illuminate - Has a positive cough reflex
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How do you manage a hernia in a child?
- Most hernias can be successfully reduced by taxis (gentle compression in the line of the inguinal canal) - If reduction is impossible then emergency surgery is required to reduce the risk of compromise to bowel, testes or ovaries
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What is inflammatory bowel disease?
An umbrella term for the two main diseases causing inflammation of the GI tract.
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What are the two main causes of IBD?
Ulcerative colitis and Crohn's disease
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What is Crohn's diease?
Transmural inflammation of the GI tract (anywhere from mouth to anus).
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Where is the most commonly affected area of Crohn's?
Terminal ileum and Colon
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Describe the epidemiology of Crohn's?
- Has a peak onset in early life (20-40 years). - More common in female than male
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What genetic mutations can cause Crohn's?
CARD15 and NOD2 mutation
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What is the endoscopic appearance of Crohn's?
Cobblestone appearance
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What are the signs of Crohn's?
- Abdominal tenderness - Fever - Mouth ulcers - Rectal examination will show blood, skin tags, fissures and fistulas
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What are the symptoms of Crohn's?
- Weight loss - Diarrhoea - Abdominal pain (most common in RLQ where the ileum is) - Lethargy and malaise also symptom
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What are the investigations for Crohn's?
- Faecal calprotectin (released by intestines when inflamed) - C- reactive protein is a good indication of current inflammation
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What is the diagnostic investigation for Crohn's and what will it show?
Colonoscopy- will show mucosal inflammation (deep ulcers, skip lesions and cobblestone appearance) Histology will show transmural inflammation with granulomas and goblet cells
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What are some immunosuppressants used to treat Crohn's? Levels of what should be measured before using them?
- Azathioprine - Methotrexate - Mercaptopurine Levels of **Thiopurine methyltransferase** should be measured before using
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What are the biological therapies used to treat Crohn's?
Infliximab and Adalimumab
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How would you maintain remission in Crohn's?
- First line= Azathioprine or mercaptopurine - Second line- Methotrexate with Infliximab
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When and what surgery could be used to treat Crohn's?
- When distal ileum is inflamed can surgically resect the area to prevent flare ups - Also used to treat strictures and fistulas
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What is Ulcerative colitis?
A type of IBD that typically involves the rectum and variable lengths of the colon. Will never spread beyond the ileocecal valve
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Describe the epidemiology of UC?
- Bimodal peak at 15-25 and 55-70 - More common in non-smokers
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What is the gene implicated in UC?
HLA-B27
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What are some other risk factors for UC?
- Non smoker - NSAIDs - Chronic stress/depression
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Describe the pathophysiology of UC?
- Ulcers tend to form along the inner surface or lumen of the large intestine and rectum - Thought to be autoimmune in nature cytotoxic T cells attack the lining of the colon - Also thought that patients have higher proportion of gut bacteria that release sulphides
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What antibodies are found in patients with UC?
p-ANCAs -perinuclear antineutrophilic cytoplasmic antibodies) in their blood - antibodies that target antigens in the body’s own neutrophil
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What are the signs of UC?
- Abdominal tenderness - Fever - in acute UC - Tachycardia - in acute severe UC - Fresh blood on rectal examination
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What are some cutaneous signs of UC?
- Erythema nodosum - inflammatory disorder affecting subcutaneous fat. - Pyoderma gangrenosum - rapidly enlarging, very painful ulcer.
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What are the symptoms of UC?
- Diarrhoea - Blood and mucus in stool - LLQ pain - Weight loss - Cramping rectal pain
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What investigations would you do for UC?
- Faecal calprotectin - LFT to screen for PSC - Raised C-reactive protein
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What is the gold standard for UC and what would it show?
Colonoscopy and biopsy Would show: -**shallow ulceration** - No inflammation beyond submucosa - Crypt abscesses - Goblet cell depletion
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What is the scoring system used to test the severity of UC?
Truelove and Witts' severity index
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What is the management for inducing remission in mild UC?
- First line: Aminosalicylate (mesalazine) - Second line corticosteroid (prednisolone)
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What is the management for inducing remission in severe UC?
- First line: IV corticosteroid hydrocortisone - Second line IV ciclosporin
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How would you maintain remission in UC?
- Aminosalicylate, azathioprine, mercaptopurine -May use biological therapy e.g., Infliximab if nothing else is working
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What are the surgical options for UC?
- Removal of the colon and rectum (panproctocolectomy) - Left with ileostomy or or **J-pouch** this is where small intestine is used to make rectum Surgery will be curative
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What % of patients with UC will develop colonic adenocarcinoma?
3-5%
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What are the extra intestinal signs of IBD?
A PIE SAC - Ankylosing spondylitis (HLA B27!) - Pyoderma gangrenosum - Iritis (aka anterior uveitis) - Erythema nodosum - Sclerosing cholangitis - Aphthous ulcers / amyloidosis - Clubbing
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What areas are most affected by coeliac disease?
- The small bowel in particular the jejunum - There will be **crypt hyperplasia and atrophy of intestinal villi**
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What are the symptoms of coeliac?
- Failure to thrive - Diarrhoea - Weight loss - Mouth ulcers - Anaemia - Dermatitis herpetiformis Can also present with neurological symptoms
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What condition is often linked to coeliac?
T1DM
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What are the genes associated with coeliac?
HLA-DQ2 gene (90%) HLA-DQ8 gene
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What are the antibodies tested for in coeliac?
Tissue transglutaminase antibodies (anti-TTG) Endomysial antibodies (EMAs) Remember to calculate total IgA as it can be low which will give negative test
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What will a endoscopy show in coeliac?
Crypt hypertrophy Villous atrophy
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What is marasmus?
When a child in deficient in all macronutrients and in it the child has a wasted, wizened appearance. Oedema is not present Affected children are often withdrawn and apathetic
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Define kwashiorkor - when is it commonly seen?
kwashiorkor is a type of malnutrition caused by protein deficiency. It mainly occurs in children who are weaning off breast milk, while marasmus can develop in infants.] If your diet has a lot of carbohydrates and very little proteins, you may develop kwashiorkor Will cause swelling in the stomach often seen when children are breastfed for too long
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What is Hirschsprungs?
Is a congenital condition where nerve cells of the **myenteric plexus** are absent in the distal bowel and rectum
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What is the pathophysiology of Hirschsprungs disease?
- The absence of the parasympathetic ganglion cells. In development these nerves start higher in the GI tract and migrate down - The area of the colon that is affected will vary from patient to patient. If the entire colon is affected this is called total **colonic aganglionosis**
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What is associated with Hirschsprung’s?
- Family history - Down syndrome - Neurofibromatosis - Waardenburg syndrome - Multiple endocrine neoplasia type II
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What are the symptoms of Hirschsprung’s?
- Acute intestinal obstruction shortly after birth - Delay in passing meconium - Chronic constipation - Abdominal pain and distension - Vomiting - Poor weight gain and failure to thrive
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What is Hirschsprung-Associated Enterocolitis?
- Is inflammation and obstruction of the intestine occurring in 20% of neonates with the condition - It presents with fever, abdominal distention and diarrhoea 2-4 weeks after birth - It can lead to **toxic megacolon** and bowel perforation.
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How is Hirschsprungs diagnosed?
Rectal biopsy which will show the **absence of ganglionic cells**
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What is the management of Hirschsprungs?
Surgical resection of the aganglionic section of the bowel
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What is Intussusception?
Where the bowel "invaginates" on itself and telescopes into itself This thickens the overall size of the bowel and narrows the lumen. This leads to a palpable mass and obstruction to faeces **typically between 6 months to 2 years and more common in boys**
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What conditions can cause Intussusception?
- Concurrent viral illness - Henoch-schonlein purpura - CF - Intestinal polyps - Meckel diverticulum
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What are the key presentations of Intussusception?
- Redcurrant jelly stool - Right upper quadrant mass on palpation, this is described as sausage shaped
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What are some other presentations of Intussusception?
- Severe colicky abdominal pain - Pale lethargic and unwell child - Vomiting - Intestinal obstruction
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What is used to diagnose Intussusception?
- Ultrasound - Contrast enema
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What is used to treat Intussusception?
- Enemas can be used to reduce the Intussusception - Surgical resection
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What is Meckel diverticulum a remnant of?
- It is an outpouching or bulge in the lower part of the small intestine which contains ectopic gastric mucosa or pancreatic tissue
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What can Meckels diverticulum lead to?
The pouch releases acid like the stomach which over time can cause ulcers to develop in the small intestine These can worsen and bleed which shows as blood in the poo, it can also leak the liquid inside the small intestine into the abdomen
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Give 3 signs of Meckel diverticulum?
Generally asymptomatic, but may present with bleeding or intussusception or volvulus: 1. Severe rectal bleeding 2. Intussusception 3. Volvulus
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Give some causes that could be behind Chronic non-specific diarrhoea in small children (Previously known as toddlers diarrhoea
undiagnosed coeliac disease or excessive ingestion of fruit juice, especially apple juice. temporary cow’s milk allergy following gastroenteritis, when a trial of a cow’s milk protein free diet may be helpful. dysmotility of the gut (a form of irritable bowel syndrome) So rule out Coealiac and Cows milk allergy first
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Describe what 'Colic' is
The term ‘colic’ is used to describe a common symptom complex that occurs during the first few months of life. Paroxysmal, inconsolable crying or screaming often accompanied by drawing up of the knees and passage of excessive flatus takes place several times a day.
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What is Cow's milk protein allergy?
- A condition affecting infants and young children under 3 years - It involves hypersensitivity to the protein in cow's milk.
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What are the two types of Cow’s milk protein allergy?
- IgE mediated that involves a rapid response within 2 hours of digestion - Non IgE mediated where a reaction occurs more slowly over several days
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What is the presentation of Cow’s milk protein allergy?
- Usually presents before one year of age, it may be apparent when weaned from breast milk to formula milk or food containing milk - Can present in breast fed babies and mother is consuming dairy products
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What are the symptoms of Cow’s milk protein allergy?
- Bloating and wind - Abdo pain - Diarrhoea - Vomiting - Urticarial rash - Facial swelling - Cough - Sneezing - Watery eyes - Eczema
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How can Cow’s milk protein allergy be diagnosed?
Skin prick testing
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What is the management for Cow’s milk protein allergy?
- Breast feeding mothers to avoid dairy products - Replace formula with special hydrolysed formula - Children will outgrow it so progress up milk ladder
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What is the difference between Cow’s milk protein allergy and Cow’s milk intolerance?
No allergic symptoms but GI symptoms present
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What is biliary atresia?
A congenital condition where a section of the bile duct is either narrowed or absent resulting in **Cholestasis**
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What are the signs and symptoms of biliary atresia?
**Conjugated bilirubin** is excreted in bile therefore biliary atresia prevents the excretion of it resulting in: - Significant jaundice lasting more than 14 days in term babies and 21 days in preterm babies
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What will tests show in biliary atresia?
A high proportion of conjugated to unconjugated bilirubin
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What is the management of biliary atresia?
“Kasai portoenterostomy” involves attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches
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What is physiological jaundice in neonate?
- Babies have lots of RBCs and they are more fragile, also break down more easily - This will cause mild yellowing of skin and sclera 2-7 days of age and resolved by 10
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What is a risk of high levels of bilirubin?
Kernicterus: Now rare due to good treatment of jaundice Bilirubin crosses the blood brain barrier Excessive bilirubin causes direct damage to the central nervous system Presents with less responsive, floppy, drowsy baby with poor feeding Leads to permanent damage (cerebral palsy, learning difficulties, deafness
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What is used to treat jaundice in new-borns?
Phototherapy
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What things would you typically see in Neonatal jaundice syndrome?
Baby has intrauterine growth restriction, Jaundice, failure to thrive, itchy rash, dark urine and hepatomegaly.
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What would a liver biopsy show in neonatal hepatitis syndrome? What blood tests would you do?
Liver biopsy will show: Multinucleated giant cells & Rosette formation. This will both ↑unconjugated + ↑ conjugated bilrubin. With associated deranged LFT’s
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What is a choledochal cyst? How may they present?
They are cystic dilatations of the extrahepatic biliary system They present with neonatal jaundice or, in older children, presentation is with abdominal pain, a palpable mass, jaundice, or cholangitis
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What do you do to investigate a choledocal cyst? How do you manage it?
ultrasound or magnetic resonance cholangiopancreatography. Treatment is by surgical excision of the cyst
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Name some causes of acute liver failure in children <2 years old
Infection (most common is herpes simplex) Metabolic disease Seronegative hepatitis Drug induced Neonatal haemochromatosis
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Give some causes of vomitting in Infants due to infections
Gastro-oesophageal reflux Feeding problems Infection: * Gastroenteritis * Whooping cough (pertussis) * Meningitis Infection – including pyelonephritis, septicaemia, meningitis Appendicitis
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Give some causes of vommitng in children due, to obstruction
Intussusception * Malrotation * Volvulus * Adhesions * Foreign body – bezoar Plyoric stenosis * Atresia – duodenal, other sites Hirschprungs disease
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What are the symptoms of a UTI in children?
**Fever** may be only symptom, always exclude a UTI in a child with a fever Baby symptoms: - Lethargy - Irritability - Vomiting - Poor feeding Infant/children symptoms: - Abdo pain- suprapubic - Vomiting - Dysuria - Frequency - Incontinence
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When is diagnosis of acute pyelonephritis made?
- Temp greater than 38 - Loin pain or tenderness
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What are the two indications of a UTI?
- Nitrites- E.coli and other gram neg break down nitrates into nitrites - Leukocytes- they are white blood cells normally found in small quantities in the urine an increase can suggest a UTI Nitrites are a better indication of a UTI
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What is the management for a UTI in a patient under 3 months?
- They should start immediate IV antibiotics (ceftriaxone) - Have a full septic screen Consider a lumbar puncture
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What is the treatment for over months olds with a UTI?
IF symptoms of pyelonephritis or sepsis admit for IV antibiotics if not use oral such as: - Trimethoprim - Nitrofurantoin - Cefalexin - Amoxicillin
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What is the risk of having pyelonephritis?
Can lead to scarring in the tissue and reduction of kidney function
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What is the criteria for USS in children with UTIs?
- Children under 6 months with **first UTI** should have an ultrasound within **6 weeks** or during if there are **recurrent or atypical** - Children with recurrent UTIs should have an abdominal ultrasound within 6 weeks - Children with atypical UTIs should have an abdominal ultrasound during the illness
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What is used to asses damage from recurrent or atypical UTIs?
DMSA scan will be performed **4-6 months** after the illness. It tells you how well the material is taken up by the kidneys and if there is any damage
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What is vesico-uteric reflux and how is it investigated?
- When urine flows back into the bladder from the ureters It is diagnosed using MCUG and managed: - Avoid constipation - Avoid full bladder - Prophylactic antibiotics - Surgical input
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According to NICE, what is the specific anitbiotics you should use to treat UTIs with Cystitis/Lower UTI Acute Pyelonephritis
Cystitis - oral antibiotic treatment should be started with trimethoprim (if there is low risk of resistance), or nitrofurantoin Acute Pyelonephritis - oral antibiotic treatment should be started, with either cefalexin, or co-amoxiclav.
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What is the treatment for UTI in children?
Infants younger tahn 3 months - 2-4 IV cefuroxime (1st gen Ceph, cant get through BBB so need to rule out meningitis). followed by oral - full septic screen, bloods, cultures, lactate Systemically well children - Treat with 3 days oral antibiotics. Advised to return if no better at 24-48hrs for reassessment - **Trimethoprim** Systemically unwell - (Fever > 38 +/- loin pain / tenderness) Treat with 7-10 days oral antibiotics (Consider IV antibiotics according to clinical judgement. Use lower threshold for IV antibiotics in younger children, those with significant risk factors and severely ill.)
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What is MCUG?
Micturating cystourethrogram (MCUG) should be used to investigate atypical or recurrent UTIs in children under 6 months. It is also used where there is a family history of vesico-ureteric reflux, dilatation of the ureter on ultrasound or poor urinary flow. A MCUG is used to diagnose VUR. It involves catheterising the child, injecting contrast into the bladder and taking a series of xray films to determine whether the contrast is refluxing into the ureters. Children are usually given prophylactic antibiotics for 3 days around the time of the investigation.
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What is enuresis?
Failure to control urination normally controlled in the day by 2 years and at night by 3-4 years
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What is primary nocturnal enuresis?
- When a child has never been consistently dry at night often a normal variation in children aged 5 and above
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What are the causes of primary nocturnal enuresis?
Overactive bladder. Frequent small volume urination prevents the development of bladder capacity. Fluid intake prior to bedtime, particularly fizzy drinks, juice and caffeine, which can have a diuretic effect Failure to wake due to particularly deep sleep and underdeveloped bladder signals Psychological distress, for example low self esteem, too much pressure or stress at home or school Secondary causes such as chronic constipation, urinary tract infection, learning disability or cerebral palsy
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What is secondary enuresis?
When a child starts wetting the bed when previously being dry for at least 6 months
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What are causes of secondary enuresis?
- UTI - Constipation - T1DM - New psychosocial problems - Maltreatment Always think about abuse and safeguarding, particularly with deliberate bedwetting, punishment for bedwetting (despite parental education) or unexplained secondary nocturnal enuresis.
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What is the pharmological treatment for enuresis?
Desmopressin is an analogue of vasopressin (also known as anti-diuretic hormone). It reduces the volume of urine produced by the kidneys. It is taken at bedtime with the intention of reducing nocturnal enuresis. Oxybutinin is an anticholinergic medication that reduces the contractility of the bladder. It can be helpful where there is an overactive bladder causing urge incontinence. Imipramine is a tricyclic antidepressant. It is not clear how it works, but it may relax the bladder and lighten sleep.
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What is the treatment for over months olds with a UTI?
IF symptoms of pyelonephritis or sepsis admit for IV antibiotics if not use oral such as: - Trimethoprim - Nitrofurantoin - Cefalexin - Amoxicillin
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What are some pre-renal causes of AKI?
Shock- due to fluid or blood loss or sepsis Decompensated liver disease- Hepatorenal syndrome GI loses
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What are some intrarenal causes of AKIs?
Vascular: **– haemolytic uraemic syndrome** - most common type of renal – vasculitis – embolus * Tubular: – acute tubular necrosis – ischaemic – toxic - drugs * Glomerular: – glomerulonephritis * Interstitial: – pyelonephritis
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What are some postrenal causes of AKI?
Obstruction - Congenital e.g. posterior urethral valves - Acquired e.g. blocked urinary catheter
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What is hepatorenal syndrome?
- Liver cirrhosis leads to portal hypertension, in response to this **portal blood vessels dilate** causing blood to pool there - This causes a lack of blood volume elsewhere including the kidneys Leads to **hypotension in the kidney and activation of the RAAS.** This causes renal vasoconstriction, which combined with low circulation volume leads to starvation of blood to the kidney *Hepatorenal syndrome is fatal within a week or so unless liver transplant is performed*
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How do you treat prerenal failure?
circulation and fluid balance meticulously monitored urgently corrected with fluid replacement and circulatory support
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Hyperkalaemia is a complication of AKI, and can lead to a cardiac arrest. How can you manage hyperkalaemia?
1. **IV stat of Calcium Gluconate** - Stabilisation of the myocardium 2. **Variable rate insulin with dextrose infusion - to increase cellular uptake of potassium** = Reduction of serum potassium 3. **oral calcium resonium** - Reduction of total body potassium - treat underlying cause
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CKD classification - outline the stages GFR 1-5 entail?
1 >90 Normal or increased GFR with other evidence of renal damage* 2 60–89 Slight decrease GFR with other evidence of renal damage* 3 A - 45–59, 3 B - 30–44 = Moderate decreased GFR with or without evidence of other renal damage 4 15–29 Severe decreased GFR with or without evidence of renal damage 5 <15 Established renal failure
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What are some features of late stage renal failure in children?
- Anorexia - Polydipsia and polyuria - Failure to grow - Bone deformities - Hypertension - Proteinuria - Normocytic anaemia
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What is nephrotic syndrome?
When the basement membrane in the glomerulus becomes highly permeable to **protein**. This allows them to leak from the blood into the urine
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What is the classic triad of nephrotic syndrome?
- Low serum albumin - High urine protein content (frothy urine) - Oedema
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What are three other features in nephrotic syndrome?
- Deranged lipid profile: high cholesterol and triglycerides - High BP - Hyper-coagulability
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What is the most common cause nephrotic syndrome?
Most common cause is **minimal change disease** which causes over 90% of cases in children under
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What are some other causes of nephrotic syndrome?
**Intrinsic kidney disease**; - Focal segmental glomerulosclerosis - Membranoproliferative glomerulonephritis **Systemic illness** - HSP - Diabetes - Infection
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What is the management for nephrotic syndrome?
High dose steroids (i.e. prednisolone) Low salt diet Diuretics may be used to treat oedema Albumin infusions may be required in severe hypoalbuminaemia Antibiotic prophylaxis may be given in severe cases
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What is the regime for steroid treatment for nephrotic syndrome?
High dose steroids are given for 4 weeks and then gradually weaned over the next 8 weeks: 80% of children will respond to steroids, and are referred to as steroid sensitive 80% of steroid sensitive patients will relapse at some point and need further steroids Patients that struggle to wean steroids due to relapses are referred to as steroid dependant Patients that do not respond to steroids are referred to as steroid resistant In steroid resistant children, ACE inhibitors and immunosuppressants such as cyclosporine, tacrolimus or rituximab may be used.
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What are some complications of nephrotic syndrome?
- Hypovolaemia - Thrombosis - Infection - Acute or chronic renal failure - Relapse
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What is nephritic syndrome?
Refers to inflammation within the nephrons of the kidneys causing: - Reduction in kidney function - Haematuria - Proteinuria
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What are the two main causes of nephritic syndrome?
Post-streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease).
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What is a typical case of Post-Streptococcal Glomerulonephritis?
Symptoms 1-3 weeks after a beta haemolytic streptococcus infection such as tonsillitis Management is supportive but may need treatment for hypertension
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What is IgA nephropathy?
IgA nephropathy is also known as Berger’s disease. This condition is related to Henoch-Schonlein Purpura, which is an IgA vasculitis. IgA deposits in the nephrons of the kidney causes inflammation (nephritis). When a renal biopsy is taken the histology will show “IgA deposits and glomerular mesangial proliferation”. It usually presents in teenagers or young adults. Management involves supportive treatment of the renal failure and immunosuppressant medications such as steroids and cyclophosphamide to slow the progression of the disease.
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What is Henoch-Schonlein Purpura?
It is a IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children. It occurs due to IgA deposits in blood vessels
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What commonly triggers HSP?
Upper airway or GI infection and is most common in children under 10
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What are the 4 classic features of HSP>
Purpura (100%), Joint pain (75%), Abdominal pain (50%) Renal involvement (50%)
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Where is affected by the rash in HSP?
Legs and buttocks
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What joints are most affected in HSP?
Knees and ankles
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What are the GI symptoms of HSP?
Abdominal pain is indicative of gastrointestinal involvement. In severe cases, it can lead to gastrointestinal haemorrhage, intussusception and bowel infarction.
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What are the investigations for HSP?
Rule out meningococcal septicaemia leukaemia, Idiopathic thrombocytopenic purpura and haemolytic uraemic syndrome - There are many different sets of criteria for diagnosing HSP, the most recent being the EULAR/PRINTO/PRES criteria from 2010. This requires the patient to have palpable purpura (not petichiae) + at least one of: Diffuse abdominal pain Arthritis or arthralgia IgA deposits on histology (biopsy) Proteinuria or haematuria
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What is hypospadias?
A condition affecting males where the opening of the urethra is abnormally displaced towards the underside of the penis. Can also be displaced down the shaft of the penis There can also be an associated condition called chordee, where the head of the penis bends downwards.
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What are the complications of Hypospadias ?
Difficulty urinating Cosmetic and psychological concerns Sexual dysfunction
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What is Haemolytic uraemic syndrome?
It involves thrombosis in small blood vessels throughout the body triggered by shiga toxins from **E.coli or shigella**
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What increases the risk of HUS in someone with a recent episode of gastroenteritis?
Antibiotics and anti-motility medication (e.g., loperamide)
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What is the classic triad of HUS?
Microangiopathic haemolytic anaemia Acute kidney injury Thrombocytopenia
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Describe the pathophysiology of HUS?
The formation of blood clots consumes platelets, leading to thrombocytopenia. The blood flow through the kidney is affected by thrombi and damaged red blood cells, leading to acute kidney injury. Microangiopathic haemolytic anaemia (MAHA) involves the destruction of red blood cells (haemolysis) due to pathology in the small vessels (microangiopathy). Tiny blood clots (thrombi) partially obstruct the small blood vessels and churn the red blood cells as they pass through, causing them to rupture
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What is the classic presentation of HUS?
Diarrhoea which turns bloody within 3 days. Around a week after the onset of diarrhoea features of HUS will occur
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What are the symptoms of HUS?
- Fever - Abdo pain - Lethargy - Pallor - Reduced urine output - Haematuria - hypertension - Bruising - Jaundice (due to haemolysis) - Confusion
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What is the management of HUS?
Stool culture is used to establish the causative organism. HUS is a medical emergency and requires hospital admission and supportive management with treatment of: Hypovolaemia (e.g., IV fluids) Hypertension Severe anaemia (e.g., blood transfusions) Severe renal failure (e.g., haemodialysis)
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What are the inactivated vaccines?
Polio Flu vaccine Hepatitis A Rabies
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What are the subunit/conjugate vaccines?
Pneumococcus Meningococcus Hepatitis B Pertussis (whooping cough) Haemophilus influenza type B Human papillomavirus (HPV) Shingles (herpes-zoster virus)
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What are the live vaccines?
Measles, mumps and rubella vaccine: contains all three weakened viruses BCG: contains a weakened version of tuberculosis Chickenpox: contains a weakened varicella-zoster virus Nasal influenza vaccine (not the injection) Rotavirus vaccine
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What are the vaccine at 8 weeks?
6 in 1 vaccine (diphtheria, tetanus, pertussis, polio, haemophilus influenzae type B (Hib) and hepatitis B) Meningococcal type B Rotavirus (oral vaccine)
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What are the vaccines given at 12 weeks?
6 in 1 vaccine (again) Pneumococcal (13 different serotypes) Rotavirus (again)
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What are the vaccines given at 16 weeks?
6 in 1 vaccine (again) Meningococcal type B (again)
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What are the vaccines given at 1 year?
2 in 1 (haemophilus influenza type B and meningococcal type C) Pneumococcal MMR Meningococcal type B
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What vaccine is given yearly from ages 2-8?
Influenza virus (nasal vaccine)
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What vaccine is given at 3 years and 4 months?
4 in 1 (diphtheria, tetanus, pertussis and polio) MMR vaccine (again)
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What vaccine is given at 12-13 years?
Human papillomavirus (HPV) vaccine (2 doses given 6 to 24 months apart)
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What vaccine is given at 14 years?
3 in 1 (tetanus, diphtheria and polio) Meningococcal groups A, C, W and Y
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