Zero to final paeds Flashcards
(561 cards)
1
Q
What is bronchioloitis?
A
Describes inflammation and infection in the bronchioles the small airways of the lungs
2
Q
What is the most common cause of bronchiolitis?
A
RSV
3
Q
What are the sounds heard in lungs on bronchiolitis?
A
Harsh breath sounds
Wheeze
Crackles
4
Q
What is the presentation of bronchiolitis?
A
Coryzal symptoms Signs of respiratory distress Dysponea Tachyponea Poor feeding Mild fever Aponeas Wheeze and crackles
5
Q
Signs of respiratory distress?
A
Raised respiratory rate Use of accessory muscles of breathing such as sternocleidomastoid, abdominal and intercostal muscles Intercostal and subcostal recession Nasal flaring Head bobbing Tracheal tugging Cyanosis
6
Q
What is wheezing?
A
Whistling sound caused by narrowed airways typically heard during expiration
7
Q
What is grunting?
A
Caused by exhaling with the glottis partially closed to increase positive end expiratory pressuer
8
Q
What is stridor?
A
High pitched inspiratory noise caused by obstruction of the upper airway for example in croup
9
Q
What is the typical course of RSV?
A
Bronchiolitis usually starts as an upper respiratory tract infection (URTI) with coryzal symptoms. From this point around half get better spontaneously. The other halfdevelop chest symptoms over the first 1-2 days following the onset of coryzal symptoms. Symptoms are generally at their worst on day 3 or 4. Symptoms usually last 7 to 10 days total and most patients fully recover within 2 - 3 weeks.
10
Q
Reasons for admission with beonchiolitis?
A
Aged under 3 months or any pre-existing condition such as prematurity, Down’s syndrome or cystic fibrosis • 50 - 75% or less of their normal intake of milk • Clinical dehydration • Respiratory rate above 70 • Oxygen saturations below 92% • Moderate to severe respiratory distress, such as deep recessions or head bobbing • Apnoeas • Parents not confident in their ability to manage at home or difficulty accessing medical help from home
11
Q
What is the management of bronchiolotisi?
A
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
12
Q
What can saline nasal drops and nasal suctioning do?
A
Clear nasal secretions
13
Q
What is continous positive airway pressure?
A
Involves using a sealed nasal cannula
Deliver high and controlled pressure
14
Q
Signs of poor ventrilation in children?
A
Raised pCO2
Falling pH
15
Q
What is palivizumab?
A
Monoclonal antibody that targets respiratory syncyctial virus
Monthly injection is given as prevention against bronchiolotitis caused by RSV
Given to high risk babies
16
Q
What is a viral induced wheeze?
A
acute wheezy illness caused by a viral infection.
17
Q
Features of VIW
A
• Presenting before 3 years of age • No atopic history • Only occurs during viral infections
18
Q
Presentation of VIW?
A
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 4). Kindle Edition.
19
Q
Presentation of acute asthma?
A
Acute asthma presents with rapidly worsening symptoms of: • Shortness of breath • Signs of respiratory distress • Fast respiratory rate (tachypnoea) • Expiratory wheeze on auscultation heard throughout the chest • The chest can sound “tight” on auscultation, with reduced air entry
Watchman, Thomas. Zero to Finals Paediatrics (p. 4). Kindle Edition.
20
Q
What does a silent chest suggest?
A
This is where the airways are so tight it is not possible for the child to move enough air through the airways to create a wheeze. This might be associated with reduce respiratory effort due to fatigue. A less experienced practitioner may think because there is no respiratory distress and no wheeze the child is not as unwell, however in reality a silent chest is life threatening.
Watchman, Thomas. Zero to Finals Paediatrics (p. 4). Kindle Edition.
21
Q
Staples of management in acute viral induced wheeze or asthma are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
A
Supplementary oxygen if required (i.e. oxygen saturations less than 94% or respiratory distress) • Bronchodilators (e.g. salbutamol, ipratropium and magnesium sulphate) • Steroids to reduce airway inflammation: prednisone (orally) or hydrocortisone (intravenous) • Antibiotics only if a bacterial cause is suspected (e.g. amoxicillin or erythromycin)
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
22
Q
Bronchodilators are stepped up as required:
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
A
• Inhaled or nebulised salbutamol (a beta-2 agonist) •Inhaled or nebulised ipratropium bromide (an anti-muscarinic) • IV magnesium sulphate • IV aminophylline
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
23
Q
How are mild cases of asthma managed?
A
Salbutamol inhaler via a spacer
24
Q
Moderate to severe cases require a stepwise approach working upwards until control is achieved:
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
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
IV aminophylline
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
25
What is asthma?
chronic inflammatory airway disease leading to variable airway obstruction. The smooth muscle in the airways is hypersensitive, and responds to stimuli by constricting and causing airflow obstruction.
Watchman, Thomas. Zero to Finals Paediatrics (p. 6). Kindle Edition.
26
Presentation suggesting asthma diagnosis?
• Dry cough with wheeze and shortness of breath • Episodic symptoms with intermittent exacerbations • Diurnal variability, typically worse at night and early morning • Typical triggers (see below) • A history of other atopic conditions such as eczema, hayfever and food allergies • Family history of asthma or atopy • Bilateral widespread “polyphonic” wheeze heard by a healthcare professional
Symptoms improve with bronchodilators
Watchman, Thomas. Zero to Finals Paediatrics (p. 6). Kindle Edition.
27
Asthma triggers
• Dust (house dust mites) • Animals • Cold air • Exercise • Smoke • Food allergens (e.g. peanuts, shellfish or eggs)
Watchman, Thomas. Zero to Finals Paediatrics (p. 7). Kindle Edition.
28
Asthma medical therapy in under 5
1Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
2Add a low dose corticosteroid inhaler or a leukotriene antagonist (i.e. oral montelukast)
3Add the other option from step 2.
4Refer to a specialist.
Watchman, Thomas. Zero to Finals Paediatrics (p. 7). Kindle Edition.
29
Asthma medical therapy in under 5-12
1Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
2Add a regular low dose corticosteroid inhaler
3Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response. 4Titrate up the corticosteroid inhaler to a medium dose. Consider adding: • Oral leukotriene receptor antagonist (e.g. montelukast) • Oral theophylline
5Increase the dose of the inhaled corticosteroid to a high dose 6Referral to a specialist. They may require daily oral steroids.
Watchman, Thomas. Zero to Finals Paediatrics (p. 8). Kindle Edition.
30
Asthma medical therapy in over 12 (same as adults)
1 Start a short-acting beta 2 agonist inhaler (e.g. salbutamol) as required 2 Add a regular low dose corticosteroid inhaler
3 Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response.
4 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).
5 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.
6 Add oral steroids at the lowest dose possible to achieve good control under specialist guidance
Watchman, Thomas. Zero to Finals Paediatrics (p. 8). Kindle Edition.
Watchman, Thomas. Zero to Finals Paediatrics (p. 8). Kindle Edition.
31
Cn inhaled corticosteroids reduce growth?
There is evidence that inhaled steroids can slightly reduce growth velocity and can cause a small reduction in final adult height of up to 1cm when used long term (for more than 12 months). This effect is dose dependent, meaning it is less of a problem with smaller doses.
Watchman, Thomas. Zero to Finals Paediatrics (p. 8). Kindle Edition.
32
MDI technique without a spacer:
Watchman, Thomas. Zero to Finals Paediatrics (p. 9). Kindle Edition.
• Remove the cap • Shake the inhaler (depending on the type) • Sit or stand up straight • Lift the chin slightly • Fully exhale • Make a tight seal around the inhaler between the lips • Take a steady breath in whilst pressing the canister • Continue breathing in for 3 - 4 seconds after pressing the canister • Hold the breath for 10 seconds or as long as comfortably possible • Wait 30 seconds before giving a further dose • Rinse the mouth after using a steroid inhaler
Watchman, Thomas. Zero to Finals Paediatrics (p. 9). Kindle Edition.
33
MDI technique with a spacer:
Watchman, Thomas. Zero to Finals Paediatrics (p. 9). Kindle Edition.
• Assemble the spacer • Shake the inhaler (depending on the type) • Attach the inhaler to the correct end • Sit or stand up straight • Lift the chin slightly • Make a seal around the spacer mouthpiece or place the mask over the face • Spray the dose into the spacer • Take steady
Watchman, Thomas. Zero to Finals Paediatrics (p. 9). Kindle Edition.
34
What is pneumonia?
Simple infection of lung tissue
| Inflammation fo lung tissue and sputum in the airways and alveoli
35
Presentation of pneumonia
Cough (typically wet and productive) • High fever (> 38.5ºC) • Tachypnoea • Tachycardia • Increased work of breathing • Lethargy • Delirium (acute confusion associated with infection)
Watchman, Thomas. Zero to Finals Paediatrics (pp. 9-10). Kindle Edition.
36
These can indicate sepsis secondary to the pneumonia:
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
Tachypnoea (raised respiratory rate) • Tachycardia (raised heart rate) • Hypoxia (low oxygen)
Hypotension (shock) • Fever
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
37
The characteristic chest signs of pneumonia are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
• Bronchial breath sounds. These are harsh breath sounds that are equally loud on inspiration and expiration. These are caused by consolidation of the lung tissue around the airway. • Focal coarse crackles caused by air passing through sputum, similar to using a straw to blow into a drink • Dullness to percussion due to lung tissue collapse and/or consolidation
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
38
Most common bacterial cause of pneumonia?
Streptococcus pneumonia
39
Most common viral cause of pneumonia?
RSV
40
Who does group B strep pneumonia occur in?
pre-vaccinated infants, often contracted during birth from GBS colonising the vagina
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
41
What is impaging staph aureus pneumonia look like?
This causes typical chest xray findings of pneumatoceles (round air filled cavities) and consolidations in multiple lobes.
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
42
Who does haemophilus influenza pneumonia occur in?
This particularly affects pre-vaccinated or unvaccinated children.
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
43
Investigations for pneumonia?
A chest xray is the investigation of choice for diagnosing pneumonia. It is not routinely required, but can be useful if there is diagnostic doubt or in severe or complicated cases. Sending sputum cultures and throat swabs for bacterial cultures and viral PCR can establish the causative organism and guide treatment. All patients with sepsis should have blood cultures. Capillary blood gas analysis can be helpful in assessing or monitoring respiratory or metabolic acidosis and the blood lactate level in unwell patients.
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
44
Pneumonia management
Amoxicillin is often used first line. Adding a macrolide (erythromycin, clarithromycin or azithromycin) will cover atypical pneumonia. Macrolides can be used as monotherapy in patients with a penicillin allergy.
IV antibiotics can be used when there is sepsis or a problem with intestinal absorption. Oxygen is used as required to maintain saturations above 92%.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 10-11). Kindle Edition.
45
What is croup?
Acute infective respiratory disease affecting young children
46
Who does croup typically affect?
Children 6 months to 2 years but can be older
47
What does croup cause?
Oedema in the larynx
48
What is the classic cause of croup?
Parainfluenza virus
49
Common causes of croup?
• Parainfluenza • Influenza • Adenovirus • Respiratory syncytial virus (RSV)
Watchman, Thomas. Zero to Finals Paediatrics (p. 11). Kindle Edition.
50
Why is croup not caused by diptheria commonly anymore?
Vaccination
51
Presentation of croup
* Increased work of breathing • “Barking” cough, occurring in clusters of coughing episodes • Hoarse voice
* Stridor • Low grade fever
Watchman, Thomas. Zero to Finals Paediatrics (p. 11). Kindle Edition.
52
How are most cases of croup managemd?
Most cases can be managed at home with simple supportive treatment (fluids and rest). During attacks it can help to sit the child up and comfort them. Measures should be taken to avoid spreading infection, for example hand washing and staying off school.
Watchman, Thomas. Zero to Finals Paediatrics (p. 12). Kindle Edition.
53
Stepwise options in severe croup to get control of symptoms are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 12). Kindle Edition.
Oral dexamethasone • Oxygen • Nebulised budesonide • Nebulised adrenalin • Intubation and ventilation
Watchman, Thomas. Zero to Finals Paediatrics (p. 12). Kindle Edition.
54
What is epiglottitis?
Inflammation and swelling of the epiglottis
| Typically with heaemophilus influenza B
55
Why is epiglottisis now rare?
Due to routine vaccination program
56
Presentation suggesting possible epiglottitis?
• Patient presenting with a sore throat and stridor • Drooling • Tripod position, sat forward with a hand on each knee • High fever • Difficulty or painful swallowing • Muffled voice • Scared and quiet child • Septic and unwell appearance
Watchman, Thomas. Zero to Finals Paediatrics (p. 12). Kindle Edition.
57
Management of epiglottisis?
```
Emergency
Immediate risk of airway closing
Dont examine
Alert most senior paediatrician and anaesthetist available
Ensure airway is secure
Prepare in case need intubation
Also may need tracheostomoty
```
Once airway secure: IV abx, steroids
58
What is a common complication of epiglottisi?
Epiglottic abscess
Collection of pus around the epiglottis
Threatens the airways making it a life threateneing emergencu
59
What is laryngomalacia?
condition affecting infants, where the part of the larynx above the vocal cords (the supraglottic larynx) is structured in a way that allows it to cause partial airway obstruction. This leads to a chronic stridor during inspiration, when the larynx flops across the airway as the infant breathes in.
Watchman, Thomas. Zero to Finals Paediatrics (p. 13). Kindle Edition.
60
What is stridor?
harsh whistling sound caused by air being forced through an obstruction of the upper airway.
Watchman, Thomas. Zero to Finals Paediatrics (p. 13). Kindle Edition.
61
What is the presentation of laryngomalacia?
Infants
Peaking at 6 months
Presents with inspiratory strifor, harsh whistling sound when breathing in
in. Usually this is intermittent and become more prominent when feeding, upset, lying on their back or during upper respiratory tract infections. Infants with laryngomalacia do not usually have associated respiratory distress. It can cause difficulties with feeding, but rarely causes complete airway obstruction or other complications.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 13-14). Kindle Edition.
62
What is the disease course of laryngomaalcia?
The problem resolves as the larynx matures and grows and is better able to support itself, preventing it from flopping over the airway. Usually, no interventions are required and the child is monitored as they grow out of the condition. Rarely tracheostomy may be necessary. This involves inserting a tube through the front of the neck into the trachea, bypassing the larynx. Surgery is also an option to alter the tissue in the larynx and improve the symptoms.
Watchman, Thomas. Zero to Finals Paediatrics (p. 14). Kindle Edition.
63
What is whooping cough?
URTI caused by bordetella pertussis, gram negative bacteria
| Coughing fits are so severe the patient is unable to take in any air between coughs so make loud whooping sounds
64
Pertussis typically starts with what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 14). Kindle Edition.
Mild coryzal symptoms, low grade fever and possibly a mild dry cough
More severe coughing fits start after a week or more. These involve sudden and recurring attacks of coughing with cough free periods in between. This is described as a paroxysmal cough. Coughing fits are severe and keep building until the patient is completely out of breath. Patients typically produces a large, loud inspiratory whoop when the coughing ends. They can cough so hard they faint, vomit or even develop a pneumothorax. Bear in the mind that not all patients will “whoop” and infants with pertussis may present with apnoeas rather than a cough.
Watchman, Thomas. Zero to Finals Paediatrics (p. 14). Kindle Edition.
65
Diagnosis of whooping cough
A nasopharyngeal or nasal swab with PCR testing or bacterial culture can confirm the diagnosis within 2 to 3 weeks of the onset of symptoms. Where the cough has been present for more than 2 weeks, patients can be tested for the anti-pertussis toxin immunoglobulin G. This is tested for in the oral fluid of children aged 5 to 16 and in the blood of those aged over 17.
Watchman, Thomas. Zero to Finals Paediatrics (p. 14). Kindle Edition.
66
What is the management of whooping cough?
Need to notify PH
Supportive care
care. Vulnerable or acutely unwell patients, those under 6 months and patients with apnoeas, cyanosis or severe coughing fits may need to be admitted.
Measures to prevent spread are important, such as avoiding contact with vulnerable people, disposing of tissues and careful hand hygiene.
Macrolide antibiotics such as azithromycin, erythromycin and clarithromycin can be beneficial in the early stages (within the first 21 days) or vulnerable patients. Co-trimoxazole is an alternative to macrolides. Close contacts of infected patients can be given prophylactic antibiotics if they are in a vulnerable group, for example pregnant women, unvaccinated infants or healthcare workers that have contact with children or pregnant women.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 14-15). Kindle Edition.
67
What is a complication of bronchiectasis?
Whooping cough
68
What is chronic lung disease of prematurity also known as?
Bronchopulmonary dysplasia
69
Who does chronic lung disease of prematurity occur in?
Premature babies, typically born before 28 weeks gestation
70
Diagnosis of CLDP?
Diagnosis is made based on chest xray changes and when the infant requires oxygen therapy after 36 weeks gestational age.
Watchman, Thomas. Zero to Finals Paediatrics (p. 15). Kindle Edition.
71
Features of chronic lung disease of prematurity?
• Low oxygen saturations • Increased work of breathing • Poor feeding and weight gain • Crackles and wheezes on chest auscultation • Increased susceptibility to infection
Watchman, Thomas. Zero to Finals Paediatrics (p. 15). Kindle Edition.
72
How do you prevent chronic lung disease of prematurity?
There are several measure that can be taken to minimise the risk of CLDP. Giving corticosteroids (e.g. betamethasone) to mothers that show signs of premature labour at less than 36 weeks gestation can help speed up the development of the fetal lungs before birth and reduce the risk of CLDP.
Once the neonate is born the risk of CLDP can be reduced by: • Using CPAP rather than intubation and ventilation when possible • Using caffeine to stimulate the respiratory effort • Not over-oxygenating with supplemental oxygen
Watchman, Thomas. Zero to Finals Paediatrics (p. 15). Kindle Edition.
73
Management of CLDP
A formal sleep study to assess the oxygen saturations during sleep supports the diagnosis and guides management. Babies may be discharged from the neonatal unit on a low dose of oxygen to continue at home, for example 0.01 litres per minute via nasal cannula. They are followed up to slowly wean off oxygen over the first year of life. Babies with CLDP require protection against respiratory syncytial virus (RSV) to reduce the risk and severity of bronchiolitis. This involves monthly injections of a monoclonal antibody against the virus, called palivizumab. This is very expensive (around £500 per injection), and is therefore is reserved for babies meeting certain criteria.
Watchman, Thomas. Zero to Finals Paediatrics (p. 15). Kindle Edition.
74
What is cystic fibrosis?
autosomal recessive genetic condition affecting the mucus glands. It is caused by a genetic mutation of the cystic fibrosis transmembrane conductance regulatory gene on chromosome 7. There are many variants of this mutation, the most common is the delta-F508 mutation. This gene codes for cellular channels, particularly a type of chloride channel. Around 1 in 2500 children have CF and 1 in 25 are carriers of the mutation.
Watchman, Thomas. Zero to Finals Paediatrics (p. 16). Kindle Edition.
75
Key consequences of cystic fibrosis?
The key consequences of the cystic fibrosis mutation are: • Thick pancreatic and biliary secretions that cause blockage of the ducts, resulting in a lack of digestive enzymes such as pancreatic lipase in the digestive tract • Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections • Congenital bilateral absence of the vas deferens in males. Patients generally have healthy sperm, but the sperm have no way of getting from the testes to the ejaculate, resulting in male infertility
Watchman, Thomas. Zero to Finals Paediatrics (p. 16). Kindle Edition.
76
When is cystic fibrosis screened?
At birth with the newborn blood spot test
77
What is often the first sign of CF?
Meconium ileus
78
What is meconium ileus?
CF, the meconium is thick and sticky, causing it to get stuck and obstruct the bowel. This is called meconium ileus, and is practically pathognomonic for cystic fibrosis. It presents with not passing meconium within 24 hours, abdominal distention and vomiting.
Watchman, Thomas. Zero to Finals Paediatrics (p. 16). Kindle Edition.
79
Symptoms of CF
• Chronic cough • Thick sputum production • Recurrent respiratory tract infections • Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes • Abdominal pain and bloating • Parents may report the child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat • Poor weight and height gain (failure to thrive)
Watchman, Thomas. Zero to Finals Paediatrics (p. 16). Kindle Edition.
80
Signs of CF?
• Low weight or height on growth charts • Nasal polyps • Finger clubbing • Crackles and wheezes on auscultation • Abdominal distention
Watchman, Thomas. Zero to Finals Paediatrics (pp. 16-17). Kindle Edition.
81
Causes of clubbing in children?
• Hereditary clubbing • Cyanotic heart disease • Infective endocarditis • Cystic fibrosis • Tuberculosis • Inflammatory bowel disease • Liver cirrhosis
Watchman, Thomas. Zero to Finals Paediatrics (p. 17). Kindle Edition.
82
Diagnosis of CF?
• Newborn blood spot testing is performed on all children shortly after birth and picks up most cases • The sweat test is the gold standard for diagnosis • Genetic testing for the CFTR gene can be performed during pregnancy by amniocentesis or chorionic villous sampling, or as a blood test after birth
Watchman, Thomas. Zero to Finals Paediatrics (p. 17). Kindle Edition.
83
Management of CF?
Chest physiotherapy several times a day is essential to clear mucus and reduce the risk of infection and colonisation • Exercise improves respiratory function and reserve, and helps clear sputum • High calorie diet is required for malabsorption, increased respiratory effort, coughing, infections and physiotherapy • CREON tablets to digest fats in patients with pancreatic insufficiency (they replace the missing lipase enzymes) • Prophylactic flucloxacillin tablets to reduce the risk of bacterial infections (particularly staph aureus) • Treat chest infections when they occur • Bronchodilators such as salbutamol inhalers can help treat bronchoconstriction • Nebulised DNase (dornase alfa) is an enzyme that can break down DNA material in respiratory secretions, making secretions less viscous and easier to clear • Nebulised hypertonic saline • Vaccinations including pneumococcal, influenza and varicella
Watchman, Thomas. Zero to Finals Paediatrics (p. 18). Kindle Edition.
84
Monitoring patients with CF?
Patients with cystic fibrosis are managed and followed up in specialist clinics, typically every 6 months. They require regular monitoring of their sputum for colonisation of bacteria like pseudomonas. They also need monitoring and screening for diabetes, osteoporosis, vitamin D deficiency and liver failure.
Watchman, Thomas. Zero to Finals Paediatrics (p. 18). Kindle Edition.
85
Primary ciliary dyskinesia (PCD) is also known as WHAT?
Watchman, Thomas. Zero to Finals Paediatrics (p. 18). Kindle Edition.
Kartagner’s syndrome.
Watchman, Thomas. Zero to Finals Paediatrics (p. 18). Kindle Edition.
86
What is primiary ciliary dyskineasia?
Autosomal recessive condition affecting the cilia of various cells in the body. It is more common in populations where there is consanguinity,
PCD causes dysfunction of the motile cilia around the body, most notably in the respiratory tract. This leads to a buildup of mucus in the lungs, providing a great site for infection that is not easily cleared. This leads to a similar respiratory presentation to cystic fibrosis, with frequent and chronic chest infections, poor growth and bronchiectasis. It also affects the cilia in the fallopian tubes of women and the tails (flagella) of the sperm in men, leading to reduced or absent fertility. There is a strong link between primary ciliary dyskinesia and situs inversus.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 18-19). Kindle Edition.
87
Kartagner’s triad describes the three key features of PCD. Not all patients will have all three features. These are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 19). Kindle Edition.
• Paranasal sinusitis • Bronchiectasis • Situs Inversus
Watchman, Thomas. Zero to Finals Paediatrics (p. 19). Kindle Edition.
88
Situs inversus is what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 19). Kindle Edition.
condition where all the internal (visceral) organs are mirrored inside the body. The heart is on the right, the stomach is on the right and the liver is on the left.
Situs inversus alone does not cause any problems, and patients can expect to live a normal life. A small number have associated congenital heart disease, such as transposition of the great arteries.
Watchman, Thomas. Zero to Finals Paediatrics (p. 19). Kindle Edition.
89
Diagnosis of primary ciliary dyskineasia?
The key investigation for establishing the diagnosis is to take a sample of the ciliated epithelium of the upper airway and examine the action of the cilia. A sample can be obtained through nasal brushing or bronchoscopy. Often several samples are required.
Watchman, Thomas. Zero to Finals Paediatrics (p. 19). Kindle Edition.
90
Medical causes of abdominal pain
• Constipation is very common • Urinary tract infection • Coeliac disease • Inflammatory bowel disease • Irritable bowel syndrome • Mesenteric adenitis • Abdominal migraine • Pyelonephritis • Henoch-Schonlein purpura • Tonsilitis • Diabetic ketoacidosis • Infantile colic There are addition causes in adolescent girls: • Dysmenorrhea (period pain) • Mittelschmerz (ovulation pain) • Ectopic pregnancy • Pelvic inflammatory disease • Ovarian torsion • Pregnancy
Watchman, Thomas. Zero to Finals Paediatrics (p. 21). Kindle Edition.
91
Surgical causes of abdominal pain
• Appendicitis causes central abdominal pain spreading to the right iliac fossa • Intussusception causes colicky non-specific abdominal pain with recurrent jelly stools • Bowel obstruction causes pain, distention, absolute constipation and vomiting • Testicular torsion causes sudden onset, unilateral testicular pain, nausea and vomiting
Watchman, Thomas. Zero to Finals Paediatrics (p. 21). Kindle Edition.
92
Red flags for serious abdominal pain?
• Persistent or bilious vomiting • Severe chronic diarrhoea • Fever • Rectal bleeding • Weight loss or faltering growth • Dysphagia (difficulty swallowing) • Nighttime pain • Abdominal tenderness
Watchman, Thomas. Zero to Finals Paediatrics (p. 21). Kindle Edition.
93
Raised inflammatory markers (ESR and CRP) can indicate what abdominal conditions?
Watchman, Thomas. Zero to Finals Paediatrics (p. 21). Kindle Edition.
IBD
94
ANAEMIA can indicate what abdominal conditions?
IBD or coeliac
95
Raised anti-TTG or anti-EMA antibodies indicate what abdominal conditions?
Coeliac disease
96
Raised faecal calprotectin indicates what abdominal conditions?
IBD
97
Positive urine disptick indicates what abdominal conditions?
UTI
98
What is abdominal migrane?
Central abdominal pain lasting more than 1 hour
99
What is abdominal migrane associated with?
```
Nausea
Anorexia
Pallor
Headache
Photophobia
Aura
```
100
Secondary causes of constipation?
Hirschsprung's disease, cystic fibrosis or hypothyroidism.
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
101
Typical features in the history and examination that suggest constipation are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
* Less than 3 stools a week • Hard stools that are difficult to pass • Rabbit dropping stools • Straining and painful passages of stools • Abdominal pain
* Holding an abnormal posture, referred to as retentive posturing • Rectal bleeding associated with hard stools • Faecal impaction causing overflow soiling, with incontinence of particularly loose smelly stools • Hard stools may be palpable in abdomen • Loss of the sensation of the need to open the bowels
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What is encopresis?
the term for faecal incontinence. This is not considered pathological until 4 years of age. It is usually a sign of chronic constipation, where the rectum becomes stretched and looses sensation. Large hard stools remain in the rectum and only loose stools are able to bypass the blockage and leak out, causing soiling.
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Other rarer causes of encopresis include:
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• Spina bifida • Hirschprung’s disease • Cerebral palsy • Learning disability • Psychosocial stress • Abuse
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There are a number of lifestyle factors that can contribute to the development and continuation of constipation:
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
* Habitually not opening the bowels • Low fibre diet • Poor fluid intake and dehydration • Sedentary lifestyle
* Faecal impaction may require a disimpaction regime with high doses of laxatives at first • Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts.
105
Gastro-oesophageal reflux is
| what?
Where the contents from the stomach reflux through the lower oesophageal sphincter into the oesophagus, throat and mouth.
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Why in babies does stomach contents reflux more easily?
In babies there is immaturity of the lower oesophageal sphincter, allowing stomach contents to easily reflux into the oesophagus. It is normal for a baby to reflux feeds, and provided there is normal growth and the baby is otherwise well this is not a problem, however it can be upsetting for parents. This usually improves as they grow and 90% of infants stop having reflux by 1 year.
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Signs of problematic reflux include:
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* Chronic cough • Hoarse cry • Distress, crying or unsettled after feeding • Reluctance to feed
* Pneumonia • Poor weight gain
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Some of the possible causes of vomiting include:
Watchman, Thomas. Zero to Finals Paediatrics (p. 25). Kindle Edition.
• Overfeeding • Gastro-oesophageal reflux • Pyloric stenosis (projective vomiting) • Gastritis or gastroenteritis • Appendicitis • Infections such as UTI, tonsillitis or meningitis • Intestinal obstruction • Bulimia
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Red flags of GORD
* Not keeping down any feed (pyloric stenosis or intestinal obstruction) • Projectile or forceful vomiting (pyloric stenosis or intestinal obstruction) • Bile stained vomit (intestinal obstruction) • Haematemesis or melaena (peptic ulcer, oesophagitis or varices) • Abdominal distention (intestinal obstruction)
* Reduced consciousness, bulging fontanelle or neurological signs (meningitis or raised intracranial pressure) • Respiratory symptoms (aspiration and infection) • Blood in the stools (gastroenteritis or cow's milk protein allergy) • Signs of infection (pneumonia, UTI, tonsillitis, otitis or meningitis) • Rash, angioedema and other signs of allergy (cow’s milk protein allergy) • Apnoeas are a concerning feature and may indicate serious underlying pathology. They need urgent assessment.
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In simple cases of GORD explanation, reassurance and practical advice is all that is needed. Advise:
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• Small, frequent meals • Burping regularly to help milk settle • Not over-feeding • Keep the baby upright after feeding (i.e. not lying flat)
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More problematic cases of GORD can justify treatment with:
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• Gaviscon mixed with feeds • Thickened milk or formula (specific anti-reflux formulas are available) • Ranitidine • Omeprazole where ranitidine is inadequate
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What is torticollis?
forceful contraction of the neck muscles causing twisting of the neck
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What is sandifer's syndrome
This is a rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants. The infants are usually neurologically normal. The key features are torticollis and dystonia
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What is dystonia?
abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
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What can happen after feeding in pyloric stenosis?
After feeding, there is increasingly powerful peristalsis in the stomach as it tries to push food into the duodenum. Eventually it becomes so powerful that it ejects the food into the oesophagus, out of the mouth and across the room. This is called “projectile vomiting”.
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How does pyloric stensois present?
typically presents in the first few weeks of life, with a hungry baby that is thin, pale and generally failing to thrive. The classic description of vomiting you should remember for your exams is “projectile vomiting”.
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Examination after feeding for pyloric stenosis?
Feeding, often the peristalsis can be seen by observing the abdomen. A firm, round mass can be felt in the upper abdomen that “feels like a large olive”. This is caused by the hypertrophic muscle of the pylorus.
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Blood gas analysis in pyloric stensosi?
Blood gas analysis will show a hypochloric (low chloride) metabolic alkalosis as the baby is vomiting the hydrochloric acid from the stomach.
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Management of pyloric stenosis?
Diagnosis is made using an abdominal ultrasound to visualise the thickened pylorus.
Treatment involves a laparoscopic pyloromyotomy (known as “Ramstedt’s operation”). An incision is made in the smooth muscle of the pylorus to widen the canal, allowing food to pass from the stomach to the duodenum as normal.
Prognosis is excellent following the operation.
120
What is acute gastritis?
is inflammation of the stomach and presents with nausea and vomiting.
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What is enteritis?
inflammation of the intestines and presents with diarrhoea.
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What is gastroenteritis?
inflammation all the way from the stomach to the intestines and presents with nausea, vomiting and diarrhoea.
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Key conditions to think about in patients with loose stools are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
• Infection (gastroenteritis) • Inflammatory bowel disease • Lactose intolerance • Coeliac disease • Cystic fibrosis • Toddler’s diarrhoea • Irritable bowel syndrome • Medications (e.g. antibiotics)
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What does steatorrhoea suggest?
CF
125
Viral gastroenteritis is common. It is highly contagious. Common causes are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
Rotavirus
| Norovirus
126
E. coli 0157 produces what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
Shiga toxin. This causes abdominal cramps, bloody diarrhoea and vomiting.
Destroys blood cells and leads to HUS
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Symptoms of campylobacter jejuni?
a common cause of travellers diarrhoea. It is the most common bacterial cause of gastroenteritis worldwide. Campylobacter means “curved bacteria”. It is a gram negative bacteria that has a curved or spiral shape. It is spread by: • Raw or improperly cooked poultry • Untreated water • Unpasteurised milk
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How is shigella spread?
by faeces contaminated drinking water, swimming pools and food. The incubation period is 1 to 2 days and symptoms usually resolve within 1 week without treatment. It causes bloody diarrhoea, abdominal cramps and fever. Shigella can produce the Shiga toxin and cause haemolytic uraemic
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What is salmonella?
spread by eating raw eggs or poultry, or food contaminated with the infected faeces of small animals. Incubation is 12 hours to 3 days and symptoms usually resolve within 1 week. It causes watery diarrhoea that can be associated with mucus or blood, abdominal pain and vomiting.
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How is bacillus cereus spread?
a gram positive rod spread through inadequately cooked food. It grows well on food not immediately refrigerated after cooking. The typical food is fried rice left out at room temperature.
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What is yersinia?
a gram negative bacillus. Pigs are key carriers of Yersinia, and eating raw or undercooked pork can cause infection. It is also spread through contamination with the urine or faeces of other mammals, such as rats and rabbits. Yersinia most frequently affects children, causing watery or bloody diarrhoea, abdominal pain, fever and lymphadenopathy. Incubation is 4 to 7 days and the illness can last longer than other causes of enteritis, with symptoms lasting 3 weeks or more. Older children or adults can present with fever and right sided abdominal pain due to mesenteric lymphadenitis. This can give the impression of appendicitis.
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Staphylococcus aureus can produce what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 29). Kindle Edition.
Enterotoxins when growing on food such as eggs, dairy and meat. When eaten, these toxins cause small intestine inflammation. This causes symptoms of diarrhoea, perfuse vomiting, abdominal cramps and fever. These symptoms start within hours of ingestion and settle within 12 to 24 hours. It is not actually the bacteria causing the enteritis, but the staphylococcus enterotoxin.
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What is giardia lamblia?
a type of microscopic parasite. It lives in the small intestines of mammals. These mammals may be pets,
It releases cysts in the stools of infected mammals. The cysts contaminate food or water and are eaten, infecting a new host. This is called faecal-oral transmission. Infection may not cause any symptoms, or it may cause chronic diarrhoea. Diagnosis is made by stool microscopy. Treatment is with metronidazole.
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The are possible post-gastroenteritis complications:
Watchman, Thomas. Zero to Finals Paediatrics (p. 30). Kindle Edition.
• Lactose intolerance • Irritable bowel syndrome • Reactive arthritis • Guillain–Barré syndrome
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What is coeliac disease?
autoimmune condition where exposure to gluten causes an immune reaction that creates inflammation in the small intestine. It usually develops in early childhood but can start at any age.
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What are autoantibodies created in response to in coeliac disease?
Gluten
137
What do the autoantibodies target in coeliac disease
the epithelial cells of the intestine and lead to inflammation. There are two antibodies to remember: anti-tissue transglutaminase (anti-TTG) and anti-endomysial (anti-EMA) antibodies. These antibodies correlate with disease activity and will rise with more active disease and may disappear with effective treatment.
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What does coeliac disease do to small bowel?
causes atrophy of the intestinal villi. The intestinal cells have villi on them that help with absorbing nutrients from the food passing through the intestine. The inflammation causes malabsorption of nutrients and disease related symptoms.
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Symptoms of coeliac disease?
```
Failure to thrive
Diarrhoea
Fatigue
Weight loss
Mouth ulcers
Anaemia secondary to iron deficiency
Dermattis herpetiforms
```
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Genes associated with coeliac disease
• HLA-DQ2 gene (90%) • HLA-DQ8 gene
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What should all patients with type 1 diabetes be checked for?
Coeliac disease
142
What autoantibodies are associated with coeliac diserase?
• Tissue transglutaminase antibodies (anti-TTG) • Endomysial antibodies (EMAs) • Deaminated gliadin peptides antibodies (anti-DGPs)
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How is coeliac disease diagnosed?
Investigations must be carried out whilst the patient remains on a diet containing gluten, otherwise it may not be possible to detect the antibodies or inflammation in the bowel. Check the total immunoglobulin A levels to exclude IgA deficiency before checking for coeliac disease specific antibodies: • Raised anti-TTG antibodies (first choice) • Raised anti-endomysial antibodies Endoscopy and intestinal biopsy show: • “Crypt hypertrophy” • “Villous atrophy”
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What is coeliac disease associated with?
• Type 1 diabetes • Thyroid disease • Autoimmune hepatitis • Primary biliary cirrhosis • Primary sclerosing cholangitis • Down’s syndrome
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Complications of untreated coeliac disease?
• Vitamin deficiency • Anaemia • Osteoporosis • Ulcerative jejunitis • Enteropathy-associated T-cell lymphoma (EATL) of the intestine
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Features of crohn's disease?
Crohn's (crows NESTS) • N - No blood or mucus (these are less common in Crohn’s) • E - Entire GI tract • S - “Skip lesions” on endoscopy • T - Terminal ileum most affected and Transmural (full thickness) inflammation • S - Smoking is a risk factor (don’t set the nest on fire)
Crohn’s is also associated with weight loss, strictures and fistulas.
Watchman, Thomas. Zero to Finals Paediatrics (p. 32). Kindle Edition.
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Features of UC?
```
Ulcerative Colitis (remember U - C - CLOSEUP) • C - Continuous inflammation • L - Limited to colon and rectum
• O - Only superficial mucosa affected • S - Smoking is protective • E - Excrete blood and mucus • U - Use aminosalicylates • P - Primary sclerosing cholangitis
```
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Patients with inflammatory bowel disease can develop signs outside the gastrointestinal system that examiners like to test. It is worth remembering these extra-intestinal manifestations:
Watchman, Thomas. Zero to Finals Paediatrics (p. 32). Kindle Edition.
• Finger clubbing • Erythema nodosum • Pyoderma gangrenosum • Episcleritis and iritis • Inflammatory arthritis • Primary sclerosing cholangitis (ulcerative colitis)
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What is released by instestines when inflamed?
Faecal calprotectin
150
Gold standard investigation. for diagnosis of IBD?
Endoscopy
151
What is biliary atresia?
a congenital condition where a section of the bile duct is either narrowed or absent. This results in cholestasis, where the bile cannot be transported from the liver to the bowel. Conjugated bilirubin is excreted in the bile, therefore biliary atresia prevents the excretion of conjugated bilirubin.
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The initial investigation for possible biliary atresia is what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 34). Kindle Edition.
conjugated and unconjugated bilirubin. A high proportion of conjugated bilirubin suggests the liver is processing the bilirubin for excretion (by conjugating it), but is not able to excrete the conjugated bilirubin because it cannot flow through the biliary duct into the bowel.
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Management of biliary atresia
Watchman, Thomas. Zero to Finals Paediatrics (p. 34). Kindle Edition.
surgery. The “Kasai portoenterostomy” involves attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches. This is somewhat successful and can clear the jaundice and prolong survival. Often patients require a full liver transplant to resolve the condition.
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Causes of intestinal obstruction
• Meconium ileus • Hirschsprung’s disease • Oesophageal atresia • Duodenal atresia • Intussusception • Imperforate anus • Malrotation of the intestines with a volvulus • Strangulated hernia
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Presentation of intestinal obstruction
• Persistent vomiting. This may be bilious, containing bright green bile. • Abdominal pain and distention • Failure to pass stools or wind
No bowel sounds
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Inital investigation for intestinal obstruction
The initial investigation of choice is an abdominal xray. This may show dilated loops of bowel proximal to the obstruction and collapsed loops of bowel distal to the obstruction. There will also be absence of air in the rectum.
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Management of intestinal obstruction?
Patients presenting with intestinal obstruction need to be referred to a paediatric surgical unit as an emergency. Initial management involves making them nil by mouth and inserting a nasogastric tube to help drain the stomach and stop the vomiting. They will also require IV fluids to correct any dehydration and electrolyte imbalances, and keep them hydrated while waiting for definitive management of the underlying cause.
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What is Hrischprung's disease?
a congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum.
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What is myenteric plexus?
also known as Auerbach’s plexus, forms the enteric nervous system. It is the brain of the gut. This nerve plexus runs all the way along the bowel in the bowel wall, and is a complex web of neurones, ganglion cells, receptors, synapses and neurotransmitters. It is responsible for stimulating peristalsis of the large bowel. Without this stimulation the bowel loses it’s motility and stops being able to pass food along its length.
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What is the key pathophysiology of Hirschprung's disease?
Absence of parasympathetic ganglion cells. During fetal development these cells start higher in the GI tract and gradually migrate down to the distal colon and rectum. Hirschsprung’s occurs when the parasympathetic ganglion cells do not travel all the way down the colon, and a section of colon at the end is left without these parasympathetic ganglion cells.
The length of colon without innervation varies between patients from a small area to the entire colon. When the entire colon is affected, this is called total colonic aganglionosis. The aganglionic section of colon does not relax, causing it to becomes constricted. This leads to loss of movement of faeces and obstruction in the bowel. Proximal to the obstruction the bowel becomes distended and full.
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Hirschsprung’s disease usually occurs in isolation, however it is associated with a number of other syndromes, including:
Watchman, Thomas. Zero to Finals Paediatrics (p. 36). Kindle Edition.
• Down’s syndrome • Neurofibromatosis • Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair) • Multiple endocrine neoplasia type II
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It can present with acute intestinal obstruction shortly after birth or more gradually developing symptoms of:
Watchman, Thomas. Zero to Finals Paediatrics (p. 36). Kindle Edition.
• Delay in passing meconium (more than 24 hours) • Chronic constipation since birth • Abdominal pain and distention • Vomiting • Poor weight gain and failure to thrive Hirschsprung-Associated Enterocolitis
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Hirschsprung-associated enterocolitis (HAEC) is
Watchman, Thomas. Zero to Finals Paediatrics (p. 36). Kindle Edition.
inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease. It typically presents within 2 - 4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis. It is life threatening and can lead to toxic megacolon and perforation of the bowel. It requires urgent antibiotics, fluid resuscitation and decompression of the obstructed bowel.
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Management of Hischprung's disease?
Abdominal xray can be helpful in diagnosing intestinal obstruction and demonstrating features of HAEC. Rectal biopsy is used to confirm the diagnosis. The bowel histology will demonstrates an absence of ganglionic cells. Unwell children and those with enterocolitis will require initial fluid resuscitation and management of the intestinal obstruction. IV antibiotics are required in HAEC. Definitive management is by surgical removal of the aganglionic section of bowel. Most patients will live a normal life after corrective surgery, although they can have long term disturbances in bowel function and may be left with some degree of incontinence.
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What is intussuscpetion?
a condition where the bowel “invaginates” or “telescopes” into itself. Picture the bowel folding inwards. This thickens the overall size of the bowel and narrows the lumen at the folded area, leading to a palpable mass in the abdomen and obstruction to the passage of faeces through the bowel. It typically occurs in infants 6 months to 2 years and is more common in boys.
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What is intussusceptuon associated with?
• Concurrent viral illness • Henoch-Schonlein purpura • Cystic fibrosis • Intestinal polyps • Meckel diverticulum
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Presentation of intussusception?
• Severe, colicky abdominal pain • Pale, lethargic and unwell child • “Redcurrent jelly stool” • Right upper quadrant mass on palpation. This is described as “sausage shaped” • Vomiting • Intestinal obstruction
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What does recurrent jelly stools suggest?
Intussusception
169
Diagnosis of insussception?
US or constrast enema
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Complications of intussusception?
• Obstruction • Gangrenous bowel • Perforation • Death
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What is appenditis?
Inflammation of the appendix
172
Features of appenditicits?
```
The key presenting feature of appendicitis is abdominal pain. This typically starts as central abdominal pain, that moves down to the right iliac fossa (RIF) over time and eventually becomes localised in the RIF. On palpation of the abdomen there is tenderness in McBurney’s point. This is a localised area one third the distance from the anterior superior iliac spine (ASIS) to the umbilicus.
Loss of apetite
Nausea and vomiting
Guarding
Rebound tenderness
Percussion tenderness
```
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DIAGNOSIS OF APENDITICITS?
the clinical presentation and raised inflammatory markers. Performing a CT scan can be useful in confirming the diagnosis, particularly where another diagnosis is more likely. An ultrasound scan is often used in female patients to exclude ovarian and gynaecological pathology. When a patient has a clinical presentation suggestive of appendicitis but investigations are negative, the next step is to perform a diagnostic laparoscopy to visualise the appendix directly. The surgeon can proceed to an appendicectomy during the same procedure if indicated.
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Complications of appendectomy
• Bleeding, infection, pain and scars • Damage to bowel, bladder or other organs • Removal of a normal appendix • Anaesthetic risks • Venous thromboembolism
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What is type 1 DM?
disease where the pancreas stops being able to produce insulin. What causes the pancreas to stop producing insulin is unclear. There may be a genetic component. It may be triggered by certain viruses, such as the Coxsackie B virus and enterovirus.
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When does ketogenesisi ocucur?
Ketogenesis occurs when there is an insufficient supply of glucose, and glycogen stores are exhausted, such as in prolonged fasting. The liver takes fatty acids and converts them to ketones. Ketones are water soluble fatty acids that can be used as fuel. They can cross the blood-brain barrier and be used by the brain.
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What does HbA1C look at?
Glycated haemoglobin
which is how much glucose is attached to the haemoglobin molecules inside red blood cells. This is considered to reflect the average blood glucose level over the last 3 months, because red blood cells have a lifespan of around 3 to 4 months. We measure it every 3 to 6 months to track the average blood sugar over time and determine how effective our interventions are and how well controlled the diabetes is. It requires a blood sample sent to the lab, usually in red top EDTA bottle.
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What is DKA?
```
Ketone acids (ketones) are buffered in normal patients, so the blood does not become acidotic. When underlying pathology (i.e. type 1 diabetes) causes extreme hyperglycaemic ketosis, this results in a metabolic acidosis that is life threatening. This is called diabetic ketoacidosis.
patients can develop severe hypokalaemia (low serum potassium) very quickly, and this can lead to fatal arrhythmias.
```
Watchman, Thomas. Zero to Finals Paediatrics (p. 46). Kindle Edition.
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Most dangerous aspects of DKA?
dehydration, potassium imbalance and acidosis. These are what will kill the patient. Therefore the priority is fluid resuscitation to correct the dehydration, electrolyte disturbance and acidosis. This is followed by an insulin infusion to allow the cells to start taking up and using glucose and stop producing ketones.
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Presentation of DKA?
The patient will present with symptoms of the underlying hyperglycaemia, dehydration and acidosis: • Polyuria • Polydipsia • Nausea and vomiting • Weight loss • Acetone smell to their breath • Dehydration and subsequent hypotension • Altered consciousness • Symptoms of an underlying trigger (i.e. sepsis)
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Check the local DKA diagnostic criteria for your hospital. To diagnose DKA you require:
Watchman, Thomas. Zero to Finals Paediatrics (p. 46). Kindle Edition.
• Hyperglycaemia (blood glucose > 11 mmol/l) • Ketosis (blood ketones > 3 mmol/l) • Acidosis (pH < 7.3)
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The two pillars of correcting DKA are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 46). Kindle Edition.
Correct dehydration evenly over 48 hours. This will correct the dehydration and dilute the hyperglycaemia and the ketones. Correcting it faster increases the risk of cerebral oedema. Give a fixed rate insulin infusion. This allows cells to start using glucose again. This in turn switches off the production of ketones.
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What is adrenal insufficiency?
the adrenal glands do not produce enough steroid hormones, particularly cortisol and aldosterone. Steroids are essential for life. Therefore, the condition is life threatening unless the hormones are replaced.
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Adrenal insufficiaency features in babies?
• Lethargy • Vomiting • Poor feeding • Hypoglycaemia • Jaundice • Failure to thrive
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Adrenal insufficiency features in older children?
• Nausea and vomiting • Poor weight gain or weight loss • Reduced appetite (anorexia) • Abdominal pain • Muscle weakness or cramps • Developmental delay or poor academic performance • Bronze hyperpigmentation to skin in Addison’s caused by high ACTH levels. ACTH stimulates melanocytes.
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The short synacthen test can be used to confirm
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Adrebal insufficiency
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Treatment of adrenal insufficiency is what?
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replacement steroids titrated to signs, symptoms and electrolytes.
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What is addisonian crisis
an acute presentation of severe Addison’s, where the absence of steroid hormones result in a life threatening presentation.
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Management of addisonian crisis?
• Intensive monitoring if they are acutely unwell • Parenteral steroids (i.e. IV hydrocortisone) • IV fluid resuscitation • Correct hypoglycaemia • Careful monitoring of electrolytes and fluid balance
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What is congenital adrenal hyperplasia caused by?
is caused by a congenital deficiency of the 21-hydroxylase enzyme. This causes underproduction of cortisol and aldosterone and overproduction of androgens from birth.
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Genetic inhertiance of congenital adrenal hyperplasia?
Autosomal recessive
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21-hydroxylase is the enzyme responsible for what?
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converting progesterone to aldosterone and cortisol.
Converted to testosterone instead so high testosterone, low aldosterone and low cortisol
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Female patients with CAH usually present how
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presents at birth with virilised genitalia, known as “ambiguous genitalia” and an enlarged clitoris due to the high testosterone levels.
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Congenital growth hormone deficiency results from what
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disruption to the growth hormone axis at the hypothalamus or pituitary gland. It can be due to a known genetic mutation such as the GH1 (growth hormone 1) or GHRHR (growth hormone releasing hormone receptor) genes, or due to another condition such as empty sella syndrome where the pituitary gland is under-developed or damaged.
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Growth hormone deficiency may present in neonates:
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• Micropenis (in males) • Hypoglycaemia • Severe jaundice
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Older infants and children can present with:
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• Poor growth, severely slowing from age 2 - 3 • Short stature • Slow development of movement and strength • Delayed puberty
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What is congenital hypothyrodisim>
where the child is born with an underactive thyroid gland. This occurs in around 1 in 3000 newborns. It can be the result of an underdeveloped thyroid gland (dysgenesis) or a fully developed gland that does not produce enough hormone (dyshormonogenesis). Very rarely it can be the result of a problem with the pituitary or hypothalamus. This usually occurs without any other problems and the cause is not clear.
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Congenital hypothyroidism is screened for on the newborn blood spot screening test. Where it is not picked up a birth, patients can present with:
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• Prolonged neonatal jaundice • Poor feeding • Constipation • Increased sleeping • Reduced activity • Slow growth and development
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The diagnosis of acute pyelonephritis is made if there is either:
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• A temperature greater than 38°C • Loin pain or tenderness
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What are leukocytes?
WBCs
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What does presence of nitrites suggest?
Bacteria in urine
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Management of vesico-ureteric reflux depends on the severity:
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• Avoid constipation • Avoid an excessively full bladder • Prophylactic antibiotics • Surgical input from paediatric urology
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Micturating cystourethrogram (MCUG) should be used to investigate what
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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 vulvovaginitis?
to inflammation and irritation of the vulva and vagina. It is a common condition affecting girls between the ages of 3 and 10 years. This irritation is caused by sensitive and thin skin and mucosa around the vulva and vagina in young girls. The vagina is more prone to colonisation and infection with bacteria spread from faeces.
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This irritation is caused by sensitive and thin skin and mucosa around the vulva and vagina in young girls. The vagina is more prone to colonisation and infection with bacteria spread from faeces. It can be exacerbated by:
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• Wet nappies • Use of chemicals or soaps in cleaning the area • Tight clothing that traps moisture or sweat in the area • Poor toilet hygiene • Constipation • Threadworms • Pressure on the area, for example horse riding • Heavily chlorinated pools
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Nephrotic syndrome occurs when what?
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when the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine. It is most common between the ages of 2 and 5 years. It presents with frothy urine, generalised oedema and pallor.
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Nephrotic syndrome features a classic triad of:
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• Low serum albumin • High urine protein content (more than 3+ protein on urine dipstick) • Oedema
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There are three other features that occur in patients with nephrotic syndrome:
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• Deranged lipid profile, with high levels of cholesterol, triglycerides and low density lipoproteins • High blood pressure • Hyper-coagulability, with an increased tendency to form blood clots
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Most common cause of nephrotic disease in children?
Minimal change disease
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Nephrotic disease can be secondary to intrinsic kidney disease such as
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• Focal segmental glomerulosclerosis • Membranoproliferative glomerulonephritis
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Management of minimal change disease?
corticosteroids (i.e. prednisolone). The prognosis is good and most children make a full recovery, however it may reoccur.
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Manage,emt of nephrotic syndrtome
• 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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Complications of nephrotic syndrome?
* Hypovolaemia occurs as fluid leaks from the intravascular space into the interstitial space, causing oedema and low blood pressure
* Thrombosis can occur because proteins that normally prevent blood clotting are lost in the kidneys, and because the liver responds to the low albumin by producing pro-thrombotic proteins • Infection occurs as the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond. This is exacerbated by treatment with medications that suppress the immune system, such as steroids. • Acute or chronic renal failure • Relapse
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What is nephritis?
inflammation within the nephrons of the kidneys.
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What can nephritis cause?
• Reduction in kidney function • Haematuria: invisible or visible amounts of blood in the urine • Proteinuria: although less than in nephrotic syndrome
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The two most common causes of nephritis in children
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are post-streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease).
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When does post strep glomerulonephritis occur?
1 - 3 weeks after a beta-haemolytic streptococcus infection, such as tonsillitis caused by Streptococcus pyogenes. Immune complexes made of streptococcal antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney and cause inflammation. This inflammation leads to an acute deterioration in renal function, causing an acute kidney injury.
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Management of post strep glomerulonephritis?
Management is supportive and around 80% of patients will make a full recovery. In some cases patients can develop a progressive worsening of their renal function. They may need treatment with antihypertensive medications and diuretics if they develop complications such as hypertension and oedema.
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What is IgA nephropathy also know as
Berger's disease
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What is the pathology of IgA nephropathy?
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”.
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Managment of IgA nephropathy
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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When does Haemolytic uraemiac syndrome occur?
When there is thrombosis within small blood vessels throughout the body. Usually triggered by shiga toxin
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What does HUS lead to?
leads to the classic triad of: • Haemolytic anaemia: anaemia caused by red blood cells being destroyed • Acute kidney injury: failure of the kidneys to excrete waste products such as urea • Thrombocytopenia: low platelet count
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Presentation of HUS
brief gastroenteritis, often with bloody diarrhoea. The symptoms of haemolytic uraemic syndrome typically start around 5 days after the onset of the diarrhoea.
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sIGNS AND SYMPTOMS OF HUS
• Reduced urine output • Haematuria or dark brown urine • Abdominal pain • Lethargy and irritability • Confusion • Oedema • Hypertension • Bruising
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What is enuresis?
Involuntary urination
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Management of primary noctural enuresis?
• Reassure parents of children under 5 years that it is likely to resolve without any treatment • Lifestyle changes: reduced fluid intake in the evenings, pass urine before bed and ensure easy access to a toilet • Encouragement and positive reinforcement. Avoid blame or shame. Punishment should very much be avoided. • Treat any underlying causes or exacerbating factors, such as constipation • Enuresis alarms • Pharmacological treatment
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What is secondary noctural enuresis?
where a child begins wetting the bed when they have previously been dry for at least 6 months. This is more indicative of an underlying illness than primary enuresis.
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Causes of secondary nocturnal enuresis include:
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• Urinary tract infection • Constipation • Type 1 diabetes • New psychosocial problems (e.g. stress in family or school life) • Maltreatment
ALWAYS CONSIDER ABUSE AND SAFEGUARDING
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What are enuresis alarms?
An enuresis alarm is a device that makes a noise at the first sign of bed wetting, waking the child and stopping them from urinating. It requires quite a high level of training and commitment and needs to be used consistently for a prolonged period (i.e. at least 3 months). Some families may find them very helpful, whereas others may find they add to the burden and frustration and are counterproductive.
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What is desmopressin?
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.
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What is oxybutinin?
anticholinergic medication that reduces the contractility of the bladder. It can be helpful where there is an overactive bladder causing urge incontinence.
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What is imipramine
tricyclic antidepressant. It is not clear how it works, but it may relax the bladder and lighten sleep.
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When does autosomal recessive polycystic kidney disease present?
In neonates
Picked up on antenatal US scans
scans. It is the result of a mutation in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6. This gene codes for the fibrocystin/polyductin protein complex (FPC), which is responsible for the creation of tubules and the maintenance of healthy epithelial tissue in the kidneys, liver and pancreas.
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Features of Polycystic kdiney disease?
• Cystic enlargement of the renal collecting ducts • Oligohydramnios, pulmonary hypoplasia and Potter syndrome • Congenital liver fibrosis
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What is Wilm's tumour?
Specific tumour affecting the kidneys in children, typically under 5
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Consider a Wilm’s tumour in a child under the age of 5 years presenting with a mass in the abdomen. The parents may have noticed the mass, or they may present with signs and symptoms of:
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• Abdominal pain • Haematuria • Lethargy • Fever • Hypertension • Weight loss
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What is a posterior urethral valve
where there is tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output. It occurs in newborn boys. The obstruction to the outflow of urine creates a back pressure into the bladder, ureters and up to the kidneys, causing hydronephrosis. A restriction in the outflow of urine prevents the bladder from fully emptying, leading to a reservoir of urine that increases the risk of urinary tract infections.
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Presentation of posterior urethral valve
• Difficulty urinating • Weak urinary stream • Chronic urinary retention • Palpable bladder • Recurrent urinary tract infections • Impaired kidney function
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Risk factors for undesceded testis?
• Family history of undescended testes • Low birth weight • Small for gestational age • Prematurity • Maternal smoking during pregnancy
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Undescended testes in older children or after puberty hold a higher risk of what
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of testicular torsion, infertility and testicular cancer.
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Management of undescended testis?
Watching and waiting is appropriate in newborns. In most cases the testes will descend in the first 3 - 6 months. If they have not descended by 6 months they should be seen by a paediatric urologist. Orchidopexy (surgical correction of undescended testes) should be carried out between 6 and 12 months of age.
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What is hypospadias?
a condition affecting males, where the urethral meatus (the opening of the urethra) is abnormally displaced posteriorly on the penis. This might be further towards the bottom of the glans (in 90% of cases), halfway down the shaft or even at the base of the shaft.
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What is epispadias?
is where the meatus is displaced anteriorly, on the top of the penis. Usually, the foreskin is abnormally formed to match the position of the meatus.
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Management of hypospdias?
• Mild cases may not require any treatment • Surgery is usually performed after 3 - 4 months of age • Surgery aims to correct the position of the meatus and straighten the penis
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What is a hydrocele?
a collection of fluid within the tunica vaginalis that surrounds the testes. The tunica vaginalis is a sealed pouch of membrane that surrounds the testes. Originally the tunica vaginalis is part of the peritoneal membrane, but during development of the fetus it becomes separated from the peritoneal membrane and remains in the scrotum, partially covering each testicle.
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What are simple hydroceles?
Common in newborn males. They occurs where fluid is trapped in the tunica vaginalis. Usually this fluid gets reabsorbed over time and the hydrocele disappears.
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What is communicating hydroceles?
occur where the tunica vaginalis around the testicle is connected with the peritoneal cavity via a pathway called the processus vaginalis. This allows fluid to travel from the peritoneal cavity into the hydrocele, allowing the hydrocele to fluctuate in size.
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Management of simple hydroceles?
will usually resolve within 2 years without having any lasting negative effects. Parents can be reassured and followed up routinely. They may require surgery if they are associated with other problems, such as a hernia.
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Management of communicating hydroceles?
be treated with a surgical operation to remove or ligate the connection between the peritoneal cavity and the hydrocele (the processus vaginalis).
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What is surface tension?
attraction of the molecules in a liquid to each other, pulling them together and minimising surface area. This is why, in zero gravity, water floats around in a ball rather than diffusing into a mist.
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What are alveoli?
small sacs in the lung where gas collects and diffuses into the blood during inhalation. They are lined with fluid. The molecules of this fluid pull together due to surface tension, in turn pulling the walls of the alveoli towards each-other, attempting to collapse the space in the alveoli.
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What is surfactant?
fluid produced by type II alveolar cells. It contains proteins and fats. It sits on top of the water in the lungs. It has a hydrophilic side that faces the water, and a hydrophobic side that faces the air. The surfactant reduces the surface tension of the fluid in the lungs, essentially providing a barrier that reduces the water molecules tendency to pull towards each other.
The result is that surfactant keeps the alveoli inflated and maximises the surface area of the alveoli. This reduces the force needed to expand the alveoli, and therefore the lungs, during inspiration. This is compliance
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When do type II alveolar cells start to produce surfactant? What does this mean for preterm bbies
24 and 34 weeks gestation. This means pre-term babies have problems associated with reduced pulmonary surfactant.
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What are the cardiorespiratory changes at birth?
During birth the thorax is squeezed as the body passes through the vagina, helping to clear fluid from the lungs. Birth, temperature change, sound and physical touch stimulate the baby to promote the first breath. A strong first breath is required to expand the previously collapsed alveoli for the first time. Adrenalin and cortisol are released in response to the stress of labour, stimulating respiratory effort. The first breaths the baby takes expands the alveoli, decreasing the pulmonary vascular resistance. The decrease in pulmonary vascular resistance causes a fall in pressure in the right atrium. At this point the left atrial pressure is greater than the right atrial pressure, which squashes the atrial septum and causes functional closure of the foramen ovale. The foramen ovale then structurally closes and becomes the fossa ovalis.
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What is the relationship bwettween blood oxygenation and prostaglandins?
Increased blood oxygenation causes a drop in circulating prostaglandings which cqauses closure of the ductus arteriosus to ligametnum arteriosus
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Extended hypoxia to the brain leads to what?
Hypoxic ischemic encephalopathy- Can lead to cerebral palsy
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[rinciples of neonatal resuscitation
```
Warm the baby
Calculate APGAR
Stimulate breathing
Inflation breaths
Chest compressions
```
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APGAR score
```
Appearance
Pulse
Grimmace
Activity
Respiration
```
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What can delayed clamping of umbilical cord cause?
After birth there is still a significant volume of fetal blood in the placenta. Delayed clamping of the umbilical cord provides time for this blood to enter the circulation of the baby. This is known as placental transfusion. Recent evidence indicates that in healthy babies, delaying cord clamping leads to improved haemoglobin, iron stores and blood pressure and a reduction in intraventricular haemorrhage and necrotising enterocolitis. The only apparent negative effect is an increase in neonatal jaundice, potentially requiring more phototherapy.
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What should be done in terms of vitamin K after birth?
Babies are born with a deficiency of vitamin K. Vitamin K is an important part of normal blood clotting. Standard practice is to give all neonates an intramuscular injection of vitamin K in the thigh shortly after birth. This can have the helpful side effect of stimulating the baby to cry, which helps expand the lungs. Vitamin K helps to prevent bleeding, particularly intracranial, umbilical stump and gastrointestinal bleeding. Alternatively, vitamin K can be given orally, however this takes longer to act and requires doses at birth, 7 days and 6 weeks.
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Benefits of skin to skin contact after birth?
• Helps warm the baby • Improves the mother and baby interaction • Calms the baby • Improves breast feeding
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What does blood spot screening look for?
* Sickle cell disease • Cystic fibrosis • Congenital hypothyroidism • Phenylketonuria • Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) • Maple syrup urine disease (MSUD)
* Isovaleric acidaemia (IVA) • Glutaric aciduria type 1 (GA1) • Homocystin
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What is caput succedaneum?
involves fluid (oedema) collecting on the scalp, outside the periosteum. Caput is caused by pressure to a specific area of the scalp during a traumatic, prolonged or instrumental delivery. The periosteum is a layer of dense connective tissue that lines the outside of the skull and does not cross the sutures (the gaps in the baby’s skull). The fluid is outside the periosteum, which means it is able to cross the suture lines. There is usually no, or only mild, discolouration of the skin. It does not require any treatment and will resolve within a few days.
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What is cephalohaemotoma?
collection of blood between the skull and the periosteum. It is caused by damage to blood vessels during a traumatic, prolonged or instrumental delivery. It can be described as a traumatic subperiosteal haematoma. The blood is below the periosteum, therefore the lump does not cross the suture lines of the skull.
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Neonatal sepsis is caused by
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infection in the neonatal period. It potentially results in significant morbidity and mortality for the affected infant, particularly if treatment is delayed.
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What does neonatal sepsis present with?
non-specific signs and requires a high degree of suspicion and a low threshold for starting treatment with broad spectrum antibiotics.
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Neonatal sepsis common organis,
• Group B streptococcus (GBS) • Escherichia coli (e. coli) • Listeria • Klebsiella • Staphylococcus aureus
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Risk factors for neonatal sepsis?
• Vaginal GBS colonisation • GBS sepsis in a previous baby • Maternal sepsis, chorioamnionitis or fever above 38ºC • Prematurity (less than 37 weeks) • Early (premature) rupture of membrane • Prolonged rupture of membranes (PROM)
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Clinical features of neonatal sepsis
• Fever • Reduced tone and activity • Poor feeding • Respiratory distress or apnoea • Vomiting • Tachycardia or bradycardia • Hypoxia • Jaundice within 24 hours • Seizures • Hypoglycaemia
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Red flag symptoms of neonatal sepsis?
• Confirmed or suspected sepsis in the mother • Signs of shock • Seizures • Term baby needing mechanical ventilation • Respiratory distress starting more than 4 hours after birth • Presumed sepsis in another baby in a multiple pregnancy
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Hypoxic ischaemic encephalopathy (HIE) occurs in neonates as a result of what?
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Result of hypoxia during birth
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Why does physiological haundice occur?
There is a high concentration of red blood cells in the fetus and neonate. These red blood cells are more fragile than normal red blood cells. The fetus and neonate also have less developed liver function.
Fetal red blood cells break down more rapidly than normal red blood cells, releasing lots of bilirubin. Normally this bilirubin is excreted via the placenta, however at birth the foetus no longer has access to a placenta to excrete bilirubin. This leads to a normal rise in bilirubin shortly after birth, causing a mild yellowing of the skin and sclera from 2 - 7 days of age. This usually resolves completely by 10 days. Most babies remain otherwise healthy and well.
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Causes of neonatal jaundice can be split into?
Increased production and decreased clearance of bilirubin
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Causes of increased production of bilirubin
• Haemolytic disease of the newborn • ABO incompatibility • Haemorrhage • Intraventricular haemorrhage • Cephalo-haematoma • Polycythaemia • Sepsis and disseminated intravascular coagulation • G6PD deficiency
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Causes of decreased clearance of bilirubin
• Prematurity • Breast milk jaundice • Neonatal cholestasis • Extrahepatic biliary atresia • Endocrine disorders (hypothyroid and hypopituitary) • Gilbert syndrome
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Why is physiological jaundice exagerated in premature neonates?
Immature liver
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Why does breast milk jaundice occur?
Components of breast milk inhibit the ability of the liver to process the bilirubin. Breastfed babies are more likely to become dehydrated if not feeding adequately. Inadequate breastfeeding may lead to slow passage of stools, increasing the absorption of bilirubin in the intestines. Breastfeeding should still be encouraged, as the benefits of breastfeeding outweigh the risks of breast milk jaundice. Mothers may need extra support and advice to ensure adequate breastfeeding.
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Haemolytic disease of the newborn is a cause of what
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haemolysis (red blood cells breaking down) and jaundice in the neonate. It is caused by incompatibility between the rhesus antigens on the surface of the red blood cells of the mother and fetus. The rhesus antigens on the red blood cells vary between individual. This is different to the ABO blood group system.
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Jaundice is “prolonged” when it lasts longer than would be expected in physiological jaundice. This is:
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• More than 14 days in full term babies • More than 21 days in premature babies
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Investigations for jaundice?
* Full blood count and blood film for polycythaemia or anaemia • Conjugated bilirubin: elevated levels indicate a hepatobiliary cause (biliary atresia) • Blood type testing of mother and baby for ABO or rhesus incompatibility
* Direct Coombs test (direct antiglobulin test) for haemolysis • Thyroid function, particularly for hypothyroid • Blood and urine cultures if infection is suspected. Suspected sepsis needs treatment with antibiotics. • Glucose-6-phosphate-dehydrogenase (G6PD) levels for G6PD deficiency
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Management of jaundice?
In jaundiced neonates, total bilirubin levels are monitored and plotted on treatment threshold charts. These charts are specific for the gestational age of the baby at birth. The age of the baby is plotted on the x-axis and the total bilirubin level on the y-axis. When the total bilirubin reaches the threshold on the chart, they need to be commenced on treatment to lower the bilirubin level.
Phototherapy is usually adequate to correct neonatal jaundice. Extremely high levels may require an exchange transfusion. Exchange transfusions involve removing blood from the neonate and replacing it with donor blood.
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What does phototherapy do?
converts unconjugated bilirubin into isomers that can be excreted in the bile and urine without requiring conjugation in the liver. Phototherapy involves removing clothing down to the nappy to exposure the skin and using eye patches to protect the eyes. A light-box shines UV light on the baby’s
skin. Double phototherapy involves two light boxes. Bilirubin is closely monitored during treatment. Once phototherapy is complete, a rebound bilirubin should be measured 12 – 18 hours after stopping, to ensure the levels do not rise above the treatment threshold again.
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What is kernicterus?
type of brain damage caused by excessive bilirubin levels. It is the main reason we treat neonatal jaundice to keep bilirubin levels below certain thresholds. Bilirubin can cross the blood-brain barrier. Excessive bilirubin causes direct damage to the central nervous system. Kernicterus presents with a less responsive, floppy, drowsy baby with poor feeding. The damage to the nervous system is permanent, causing cerebral palsy, learning disability and deafness. Kernicterus is now rare due to effective treatment of jaundice.
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285
What is prematurity?
Birth before 37 weeks gestation
286
What things are associated with prematurity?
• Social deprivation • Smoking • Alcohol • Drugs • Overweight or underweight mother • Maternal co-morbidities • Twins • Personal or family history of prematurity
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2 options to delay birth
• Prophylactic vaginal progesterone: putting a progesterone suppository in the vagina to discourage labour • Prophylactic cervical cerclage: putting a suture in the cervix to hold it closed
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288
Apnoea are often a sign of developing illness, such as:
Watchman, Thomas. Zero to Finals Paediatrics (p. 83). Kindle Edition.
• Infection • Anaemia • Airway obstruction (may be positional) • CNS pathology, such as seizures or haemorrhage • Gastro-oesophageal reflux
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Management of aponea
Neonatal units attach apnoea monitors to premature babies. These make a sound when an apnoea is occurring. Tactile stimulation is used to prompt the baby to restart breathing. Intravenous caffeine can be used to prevent apnoea and bradycardia in babies with recurrent episodes. Episodes will settle as the baby grows and develops.
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Abnormal development of the blood vessels in the retina can lead to what
Watchman, Thomas. Zero to Finals Paediatrics (p. 83). Kindle Edition.
scarring, retinal detachment and blindness. Treatment can prevent blindness, which is why screening is so important.
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291
Who does respiratory distress syndrome afect?
Premature neonates
Born before lung start producing adequate surfactant
Ground glass appearance
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Pathophysiology of RDS
Inadequate surfactant leads to high surface tension within alveoli. This leads to atelectasis (lung collapse), as it is more difficult for the alveoli and the lungs to expand. This leads to inadequate gaseous exchange, resulting in hypoxia, hypercapnia (high CO2) and respiratory distress.
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Management of RDS
```
Antenatal steroids (i.e. dexamethasone) given to mothers with suspected or confirmed preterm labour increase the production of surfactant and reduce the incidence and severity of respiratory distress syndrome in the baby. Premature neonates may need:
• Intubation and ventilation to fully assist breathing if the respiratory distress is severe • Endotracheal surfactant, which is artificial surfactant delivered into the lungs via an endotracheal tube • Continuous positive airway pressure (CPAP) via a nasal mask to help keep the lungs inflated whilst breathing • Supplementary oxygen to maintain oxygen saturations between 91 and 95% in preterm neonates
```
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294
Complications of RDS?
Short term complications: • Pneumothorax • Infection • Apnoea • Intraventricular haemorrhage • Pulmonary haemorrhage • Necrotising enterocolitis Long term complications: • Chronic lung disease of prematurity • Retinopathy of prematurity occurs more often and more severely in neonates with RDS • Neurological, hearing and visual impairment
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295
What is necrotising enterocolitis?
a disorder affecting premature neonates, where part of the bowel becomes necrotic. It is a life threatening emergency. Death of the bowel tissue can lead to bowel perforation. Bowel perforation leads to peritonitis and shock.
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296
The cause of necrotising enterocolitis is unclear. There are certain risk factors for developing NEC:
Watchman, Thomas. Zero to Finals Paediatrics (p. 85). Kindle Edition.
• Very low birth weight or very premature • Formula feeds (it is less common in babies fed by breast milk feeds) • Respiratory distress and assisted ventilation • Sepsis • Patient ductus arteriosus and other congenital heart disease
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297
Presentation fo necrotiting enterocolitis?
• Intolerance to feeds • Vomiting, particularly with green bile • Generally unwell • Distended, tender abdomen • Absent bowel sounds • Blood in stools
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Investigations for necrotising enterocolitis?
Blood tests: • Full blood count for thrombocytopenia and neutropenia • CRP for inflammation • Capillary blood gas will show a metabolic acidosis • Blood culture for sepsis Abdominal xray is the investigation of choice for diagnosis. This is done front-on in the supine position (lying face up). Additional views can be helpful, such as lateral (from the side with the patient on their back) and lateral decubitus (from the side with the neonate on their side). Xrays can show: • Dilated loops of bowel • Bowel wall oedema (thickened bowel walls) • Pneumatosis intestinalis is gas in the bowel wall and is a sign of NEC • Pneumoperitoneum is free gas in the peritoneal cavity and indicates perforation • Gas in the portal veins
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What is neonatal abstinence syndrome?
withdrawal symptoms that happen in neonates of mothers that used substances during pregnancy. The symptoms and management is slightly different for each substance used in pregnancy. Mothers should be encouraged and supported with cutting back, and if possible stopping, substances that can affect the pregnancy.
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Sustabnces that can cause neonatal abstinence syndrome
• Opiates • Methadone • Benzodiazepines • Cocaine • Amphetamines • Nicotine or cannabis • Alcohol • SSRI antidepressants
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301
Alcohol in early pregnancy can lead to:
Watchman, Thomas. Zero to Finals Paediatrics (p. 88). Kindle Edition.
Miscarriage
Small for dates
Preterm delivery
302
What is congenital rubella synrome caused by
maternal infection with the rubella virus during pregnancy. The risk is highest during the first 3 months of pregnancy.
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The features of congenital rubella syndrome to be aware of are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 88). Kindle Edition.
• Congenital cataracts • Congenital heart disease (PDA and pulmonary stenosis) • Learning disability • Hearing loss
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304
What can chickenpox in pregnancy lead to?
• More severe cases in the mother, such as varicella pneumonitis, hepatitis or encephalitis • Fetal varicella syndrome • Severe neonatal varicella infection if mum is infected around delivery
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What should be done if in doubt mother has had chickenpox
doubt, IgG levels for VZV can be tested. A positive IgG for VZV indicates immunity. Women that are not immune to varicella may be offered the varicella vaccine before or after pregnancy.
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306
The features of congenital CMV are:
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• Fetal growth restriction • Microcephaly • Hearing loss • Vision loss • Learning disability • Seizures
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There is a classic triad of features in congenital toxoplasmosis:
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• Intracranial calcification • Hydrocephalus • Chorioretinitis
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308
What are risk factors for SIDS
• Prematurity • Low birth weight • Smoking during pregnancy • Male baby (only slightly increased risk)
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Measures to reduce the risk of SIDS include:
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* Put the baby on their back when not directly supervised • Keep their head uncovered • Place their feet at the foot of the bed to prevent them sliding down and under the blanket • Keep the cot clear of lots of toys and blankets • Maintain a comfortable room temperature (16 - 20 ºC) • Avoid smoking. Avoid handling the baby after smoking (smoke stays on clothes). • Avoid co-sleeping, particularly on a sofa or chair
* If co-sleeping avoid alcohol, drugs, smoking, sleeping tablets or deep sleepers
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On formula feed, babies should receive around how much
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150ml of milk per kg of body weight. Preterm and underweight babies may require larger volumes. This is split between feeds every 2 - 3 hours initially, then to 4 hours and longer between feeds. Eventually babies and infants transition to feeding on demand
Watchman, Thomas. Zero to Finals Paediatrics (p. 92). Kindle Edition.
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When does weaning start?
6 months
312
Failure to thrive refers to poor physical growth and development in a child. Faltering growth is defined in the 2017 NICE guidelines on faltering growth in children as a fall in weight across:
Watchman, Thomas. Zero to Finals Paediatrics (p. 94). Kindle Edition.
• One or more centile spaces if their birthweight was below the 9th centile • Two or more centile spaces if their birthweight was between the 9th and 91st centile • Three or more centile spaces if their birthweight was above the 91st centile
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A child’s predicted height can be calculated based on their parents’ height, measured in centimetres. The formula is different for boys and girls:
Watchman, Thomas. Zero to Finals Paediatrics (p. 96). Kindle Edition.
• Boys: (mother height + fathers height + 14cm) / 2 • Girls: (mothers height + father height - 14cm) / 2
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314
Constitutional delay in growth and puberty (CDGP) is
Watchman, Thomas. Zero to Finals Paediatrics (p. 96). Kindle Edition.
considered a variation on normal development. It leads to short stature in childhood when compared with peers but normal height in adulthood. Puberty is delayed and the growth spurt during puberty lasts longer. They ultimately reach their predicted adult height.
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315
What is mendelian inheritance
This type of inheritance only occurs where the disease is caused by a single abnormal gene on one of the non-sex chromosomes (i.e. not the X or Y chromosomes). These non-sex chromosomes are called autosomes, which is why the conditions are “autosomal” dominant or recessive.
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316
What is mosaicism?
chromosomal abnormality actually happens after conception. The abnormality occurs in a portion of cells in the body and not in others. The person therefore has different genetic material in different cells in their body. Each case is unique and the effects are unpredictable.
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What is diagnostic testinf?
Diagnostic testing involves testing a fetus or a person for a suspected genetic condition. We can test a fetus for a genetic condition via amniocentesis. An example of this is antenatal testing for Down’s syndrome. Antenatal testing can have implications on the decision to continue the pregnancy. Where a specific condition is suspected, for example Turner syndrome, it is possible to test directly for that condition in a child or adult.
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What is predictive testing?
Predictive testing involves testing a person for a specific gene mutation that has implications for them in the future. Examples are the BRCA1 breast cancer gene or the gene for Huntington’s chorea.
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What is carrier testing?
Carrier testing involves testing parents or potential parents for the gene for a specific autosomal recessive condition in order to calculate the risk of passing it to their children. An example of this is testing for the cystic fibrosis gene.
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Dysmorphic featrures of downs syndrome
• Hypotonia (reduced muscle tone) • Brachycephaly (small head with a flat back) • Short neck • Short stature • Flattened face and nose • Prominent epicanthic folds • Upward sloping palpable fissures • Single palmar crease
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Epicanthic folds are what
Watchman, Thomas. Zero to Finals Paediatrics (p. 116). Kindle Edition.
Epicanthic folds are folds of skin overing the medial portion of the eye and eyelid. The palpable fissures are the gaps between the lower and upper eyelid.
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complications of downs syndrome
• Learning disability • Recurrent otitis media • Deafness. Eustachian tube abnormalities lead to glue ear and conductive hearing loss. • Visual problems such myopia, strabismus and cataracts • Hypothyroidism occurs in 10 - 20%
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What is the combined test for downs sybndrome?
the first line, most accurate and test of choice where possible. This test is performed between 11 and 14 weeks gestation. It involves combining results from ultrasound and maternal blood tests.
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The triple test is performed between 14 and 20 weeks gestation. It only involves maternal blood test results:
Watchman, Thomas. Zero to Finals Paediatrics (p. 116). Kindle Edition.
• Beta-HCG. A higher result indicates greater risk. • Alpha-fetoprotein (AFP). A lower result indicates a greater risk. • Serum oestriol (female sex hormone). A lower result indicates a greater risk.
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What is the quadruple test
performed between 14 and 20 weeks gestation. It is identical to the triple test but also includes maternal blood testing for inhibin-A. A higher inhibin-A indicates a greater risk.
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326
What is klinefelter syndrome?
47 XXY
327
Features of Klinefelter?
• Taller height • Wider hips • Gynaecomastia • Weaker muscles • Small testicles • Reduced libido • Shyness • Infertility
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What is turners syndrome
Female with single X
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Features of turner syndrome
• Short stature • Webbed neck • High arching palate • Downward sloping eyes with ptosis • Broad chest with widely spaced nipples • Cubitus valgus • Underdeveloped ovaries with reduced function • Late or incomplete puberty • Most females are infertile
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Features of Noonan syndrome
• Short stature • Broad forehead • Downward sloping eyes with ptosis • Hypertelorism (wide space between the eyes) • Prominent nasolabial folds • Low set ears • Webbed neck • Widely spaced nipples
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331
Marfan syndrome is an autosomal dominant condition affecting the gene responsible for creating what
Watchman, Thomas. Zero to Finals Paediatrics (p. 120). Kindle Edition.
Fibrillin
332
Features of marfan syndrome
• Tall stature • Long neck • Long limbs • Long fingers (arachnodactyly) • High arch palate • Hypermobility • Pectus carinatum or pectus excavatum • Downward sloping palpable fissures
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Fragile X syndrome is caused by a mutation in
Watchman, Thomas. Zero to Finals Paediatrics (p. 121). Kindle Edition.
mutation in the FMR1 (fragile X mental retardation 1) gene on the X chromosome. The FMR1 gene codes for the fragile X mental retardation protein, which plays a role in cognitive development in the brain.
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Features of fragile X
• Intellectual disability • Long, narrow face • Large ears • Large testicles after puberty • Hypermobile joints (particularly in the hands)
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335
Prada-Willi syndrome is a genetic condition caused by the loss of functional genes on the proximal arm of what chromosome?
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15
336
Features of prada willi syndrome
• Constant insatiable hunger that leads to obesity • Poor muscle tone as an infant (hypotonia) • Mild-moderate learning disability • Hypogonadism • Fair, soft skin that is prone to bruising • Mental health problems, particularly anxiety • Dysmorphic features • Narrow forehead • Almond shaped eyes • Strabismus • Thin upper lip • Downturned mouth
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337
Angelman syndrome is a genetic condition caused by loss of function of the UBE3A gene, specifically the copy of the gene that is inherited from the mother. This can be caused by a deletion on what chromsome
Watchman, Thomas. Zero to Finals Paediatrics (p. 123). Kindle Edition.
15
338
Features of angelman syndrome?
• Delayed development and learning disability • Severe delay or absence of speech development • Coordination and balance problems (ataxia) • Fascination with water • Happy demeanour • Inappropriate laughter • Hand flapping • Abnormal sleep patterns • Epilepsy • Attention-deficit hyperactivity disorder • Dysmorphic features • Microcephaly • Fair skin, light hair and blue eyes • Wide mouth with widely spaced teeth
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339
Williams syndrome is caused by a deletion of genetic material on one copy of what chrosome
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7
340
Features of williams
• Broad forehead • Starburst eyes (a star-like pattern on the iris) • Flattened nasal bridge • Long philtrum • Wide mouth with widely spaced teeth • Small chin • Very sociable trusting personality • Mild learning disability
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341
Haemolysis is a common cause of anaemia in infancy. There are a number of causes of haemolysis in a neonate:
Watchman, Thomas. Zero to Finals Paediatrics (p. 157). Kindle Edition.
• Haemolytic disease of the newborn due to ABO or rhesus incompatibility • Hereditary spherocytosis • G6PD deficiency
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What is idiopathic thrombocy topenic purpura?
Idiopathic thrombocytopenic purpura (ITP) is a condition characterised by idiopathic (spontaneous) thrombocytopenia (low platelet count) causing a purpuric rash (non-blanching rash). ITP is caused by a type II hypersensitivity reaction. It is caused by the production of antibodies that target and destroy platelets. This can happen spontaneously, or be triggered by something, such as a viral infection.
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Thalassaemia is related to what
Watchman, Thomas. Zero to Finals Paediatrics (p. 167). Kindle Edition.
genetic defect in the protein chains that make up haemoglobin. Normal haemoglobin consists of 2 alpha and 2 beta globin chains. Defects in the alpha globin chains lead to alpha thalassaemia. Defects in the beta globin chains lead to beta thalassaemia. Both conditions are autosomal recessive. The overall effect is varying degrees of anaemia, depending on the type and mutation.
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What is hereditary spheocytosis?
Hereditary spherocytosis is a condition where the red blood cells are sphere shaped, making them fragile and easily destroyed when passing through the spleen. It is the most common inherited haemolytic anaemia in Northern Europeans. It is an autosomal dominant condition.
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What is G6PD deficiency?
G6PD deficiency is a condition where there is a defect in the G6PD enzyme normally found in all cells of the body.
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Pathophysiology of G6PD enzyme deficiency?
The G6PD enzyme is responsible for helping protect cells from damage by reactive oxygen species (ROS). ROS are reactive molecules that contain oxygen, produced during normal cell metabolism and in higher quantities during stress on the cell. The G6PD enzyme is particularly important in red blood cells. A deficiency in G6PD makes cells more vulnerable to ROS, leading to haemolysis in red blood cells.
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347
What are allergens?
proteins that the immune system recognises as foreign and potential harmful, leading to an allergic immune response. These proteins are types of antigen.
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348
What are antigens?
are proteins that can be recognised by the immune system. The body will come in contact with millions of different antigens, and very few will lead to a hypersensitivity reaction. The ones that do are called allergens.
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Coombs and gell classification
Type 1: IgE antibodies to a specific allergen trigger mast cells and basophils to release histamines and other cytokines. This causes an immediate reaction. Typical food allergy reactions, where exposure to the allergen leads to an acute reaction, range from itching, facial swelling and urticaria to anaphylaxis. Type 2: IgG and IgM antibodies react to an allergen and activate the complement system, leading to direct damage to the local cells. Examples are haemolytic disease of the newborn and transfusion reactions. Type 3: Immune complexes accumulate and cause damage to local tissues. Examples are autoimmune conditions such as systemic lupus erythematosus (SLE), rheumatoid arthritis and Henoch-Schönlein purpura (HSP). Type 4: Cell mediated hypersensitivity reactions caused by T lymphocytes. T-cells are inappropriately activated, causing inflammation
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What is patch testing?
Patch testing is the most helpful test in determining whether a specific allergen is causing allergic contact dermatitis. It is not helpful for food allergies. The patient could be tested for reactions to latex, perfumes, cosmetics or plants. A patch containing the allergen is placed on the patient’s skin. The patch can either contain a specific allergen, or a grid of lots of allergens as a screening tool. After 2 - 3 days the skin reaction to the patch is assessed.
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What is skin prick testing?
A patch of skin is selected, usually on the patients forearm. Strategic allergen solutions are selected, for example peanuts, house dust mite and pollen. A drop of each allergen solution is placed at marked points along the patch of skin, along with a water control and a histamine control. A fresh needle is used to make a tiny break in the skin at the site of each allergen. After 15 minutes, the size of the wheals to each allergen are assessed and compared to the controls.
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What is RAST testing?
RAST testing measures the total and allergen specific IgE quantities in the patient’s blood sample. In a patient with atopic conditions such as eczema and asthma, the results will often come back positive for everything you test.
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What is cows milk protein allergy?
condition that affects infants and young children under 3 years. It involves hypersensitivity to the protein in cow’s milk. This may be IgE mediated, in which case there is a rapid reaction to cow’s milk, occurring within 2 hours of ingestion. It can also be non-IgE mediated, with reactions occurring slowly over several days. This is different to lactose intolerance and cow’s milk intolerance. People with cow’s milk protein allergy do not have an allergy to lactose. Lactose is a sugar, not a protein. Cow’s milk intolerance is not an allergic process and does not involve the immune system. Cow’s milk protein allergy is more common in formula fed babies and those with a personal or family history of other atopic conditions.
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Management of cows milk protein allergy
• Breast feeding mothers should avoid dairy products • Replace formula with special hydrolysed formulas designed for cow’s milk allergy
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What are hydrolysed formula
Hydrolysed formulas contain cow’s milk, however the proteins have been broken down so that they no longer trigger an immune response. In severe cases infants may require elemental formulas made of basic amino acids (e.g. neocate). Most children will outgrow cow’s milk protein allergy by age 3, often earlier.
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What is severe combined immmunodeficiency?
most severe condition causing immunodeficiency. Children with SCID have almost no immunity to infections. It is a syndrome caused by a number of different genetic disorders that result in absent or dysfunctioning T and B cells.
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What is omenn syndrme?
a rare cause of SCID. It is the result of a mutation in the recombination-activating gene (RAG 1 or RAG 2) that codes for important proteins in T and B cells. It has autosomal recessive inheritance.
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What are B cells?
responsible for producing antibodies, an essential component of the specific immune system. Abnormal B cells lead to a deficiency in immunoglobulins (antibodies). Deficiency in immunoglobulins is called hypogammaglobulinemia. Clinically, this leads to a susceptibility to recurrent infections, particularly lower respiratory tract infections.
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Common variable immunodeficiency is caused by WHAT
Watchman, Thomas. Zero to Finals Paediatrics (p. 182). Kindle Edition.
genetic mutation in the genes coding for components of B cells. The result is deficiency in IgG and IgA, with or without a deficiency in IgM. This leads to recurrent respiratory tract infections, typically leading to chronic lung disease over time. Patients are unable to develop immunity to infections or vaccinations. They are also prone to immune disorders such as rheumatoid arthritis, and cancers such as non-Hodgkins lymphoma. Management is with regular immunoglobulin infusions and treating infections and complications as they occur.
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What is X linked agammaglobulinaemia?
also known as Bruton’s agammaglobulinaemia. This is an X-linked recessive condition. It results in abnormal B cell development and deficiency in all classes of immunoglobulins. It causes similar issues to common variable immunodeficiency.
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Conditions that cause abnormal or absent T cells will result in what
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Immunodeficiency
362
Complement disorders affect what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 184). Kindle Edition.
the complement proteins that make up the complement system, which helps destroy pathogenic cells. Complement proteins are most important in dealing with encapsulated organisms, such as: • Haemophilus influenza B • Streptococcus pneumonia • Neisseria meningitidis
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What is mannose binding lectin deficiency?
Deficiency in mannose-binding lectin is relatively common in the general population. A deficiency leads to inhibition of the alternative pathway of the complement system. In otherwise healthy individuals, it seems to be relatively unimportant and does not seem to cause major immunodeficiency. In patients who are otherwise susceptible to infection (e.g. cystic fibrosis) it can lead to a more severe variant of their existing disease.
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What is neisseria meningitis?
gram negative diplococcus bacteria. They are circular bacteria (cocci) that occur in pairs (diplo-). It is commonly known as meningococcus.
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Wahat is meningococcal septicaemia?
meningococcus bacterial infection in the bloodstream. Meningococcal refers to the bacteria and septicaemia refers to infection in the blood stream. Meningococcal septicaemia is the cause of the classic “non-blanching rash” that everybody worries about. This rash indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages.
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Community management of bacterial meningitis
Children seen in the primary care setting with suspected meningitis AND a non blanching rash should receive an urgent stat injection (IM or IV) of benzylpenicillin prior to transfer to hospital, as time is so important. The dose will depending on their age. Giving antibiotics should not delay transfer to hospital. Where there is a true penicillin allergy, transfer should be the priority rather than finding alternative antibiotics.
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Hospital management of bacterial meningitis
CSF
| Meningococcal pcr if meningococcal disease is suspected
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Management of encephalitis?
Intravenous antiviral medications are used to treat the suspected or confirmed underlying cause: • Aciclovir treats herpes simplex virus (HSV) and varicella zoster virus (VZV) • Ganciclovir treat cytomegalovirus (CMV) Repeat lumbar puncture is usually performed to ensure successful treatment prior to stopping antivirals.
Aciclovir is usually started empirically in suspected encephalitis until results are available. Other viral causes have no effective treatment and management is supportive. Followup, support and rehabilitation is required after encephalitis, with help managing the complications.
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Infectious mononucleosis (IM) is
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condition caused by infection with the Epstein Barr virus (EBV). It is commonly known as the “kissing disease”, “glandular fever” or “mono”. This virus is found in the saliva of infected individuals. Infection may be spread by kissing or sharing cups, toothbrushes and other equipment that transmits saliva.
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370
Children to test for hep B
• Children of hepatitis B positive mums (screen at 12 months of age or any time after that) • Migrants from endemic areas • Close contacts of patients with hepatitis B
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371
To reduce the risk of the baby contracting hepatitis B, at birth (within 24 hours) neonates with hepatitis B positive mothers should be given both:
Watchman, Thomas. Zero to Finals Paediatrics (p. 199). Kindle Edition.
• Hepatitis B vaccine • Hepatitis B immunoglobulin infusion
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372
What is hep c
an RNA virus. It is spread by blood and bodily fluids. No vaccine is available. It is now curable in adults, using direct acting antiviral medications. These treatments are not yet available for children.
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373
What is the centor criteria?
used to estimate the probability that tonsillitis is due to bacterial infection, and will benefit from antibiotics.
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374
Centor criteria
• Fever over 38ºC • Tonsillar exudates • Absence of cough • Tender anterior cervical lymph nodes (lymphadenopathy)
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375
What is alternative to centro
FeverPAIN score
376
What is feverPAIN score
• Fever during previous 24 hours • P - Purulence (pus on tonsils) • A - Attended within 3 days of the onset of symptoms • I - Inflamed tonsils (severely inflamed) • N - No cough or coryza
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377
Complications of tonsillitis?
• Chronic tonsillitis • Peritonsillar abscess, also known as quinsy • Otitis media, if the infection spreads to the inner ear • Scarlet fever • Rheumatic fever • Post-streptococcal glomerulonephritis • Post-streptococcal reactive arthritis
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378
What is quinsy?
peritonsillar abscess. Peritonsillar abscess arises when there is a bacterial infection with trapped pus, forming an abscess in the region of the tonsils.
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379
Presentation of quinsy
• Sore throat • Painful swallowing • Fever • Neck pain • Referred ear pain • Swollen tender lymph nodes
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380
The NICE clinical knowledge summaries give the number of episodes required for a tonsillectomy:
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• 7 or more in 1 year • 5 per year for 2 years • 3 per year for 3 years
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381
The most common bacterial causes of otitis media, as well as other ENT infections such as rhino-sinusitis and tonsillitis is what
Watchman, Thomas. Zero to Finals Paediatrics (p. 206). Kindle Edition.
Streptococcus pneumonia
382
How long can otitis media nornmally last?
A week
383
Complications of otitis media
• Otitis media with effusion • Hearing loss (usually temporary) • Perforated eardrum • Recurrent infection • Mastoiditis (rare) • Abscess (rare)
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384
What is glue ear also called
Otitis media with effusion
385
What are gromments?
tiny tubes inserted into the tympanic membrane by an ENT surgeon. They allows fluid from the middle ear to drain through the tympanic membrane into the ear canal.
Watchman, Thomas. Zero to Finals Paediatrics (p. 208). Kindle Edition.
386
What is an audiogram
Audiograms are charts that document the volume at which patients can hear different tones. The frequency in hertz (Hz) is plotted on the x-axis, from low to high pitched. The volume in decibels (dB) is plotted on the y-axis, from loud at the bottom to quiet at the top. It is worth noting that the lower down the chart, the higher the decibels and the louder the volume.
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387
Where is kiesselbach's plexus?
Little's area
388
What is kiesselbach's plexus area
This is an area of the nasal mucosa at the front of the nasal cavity that contains a lot of blood vessels. When the mucosa is disrupted in this area and the blood vessels are exposed, for example due to trauma from a child picking their nose, they are prone to bleeding.
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389
Treatment options of nose bleeds?
• Nasal packing using nasal tampons or inflatable packs • Nasal cautery using silver nitrate sticks
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390
What is cleft lip?
congenital condition where there is a split or open section of the upper lip. This opening can occur at any point along the top lip, and can extend as high as the nose.
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391
What is cleft palate?
defect exists in the hard or soft palate at the roof of the mouth. This leaves an opening between the mouth and the nasal cavity. Cleft lip and cleft palate can occur together or on their own.
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392
What is tongue tie also called?
Ankylogossia
393
What is tongue tie?
Tight lingual frenulum, the attachment of the tongue to the floor of the mouth
394
What is a cystic hygroma?
malformation of the lymphatic system that results in a cyst filled with lymphatic fluid. It is most commonly a congenital abnormality and is typically located in the posterior triangle of the neck on the left side. It may be seen on antenatal scans, picked up on routine baby checks or discovered later when noticed incidentally.
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395
How does a thyroglossal cyst develop?
During fetal development, the thyroid gland starts at the base of the tongue. From here it gradually travels down the neck to the final position in front of the trachea, beneath the larynx. It leaves a track behind called the thyroglossal duct, which then disappears. When part of the thyroglossal duct persists it can give rise to a fluid filled cyst. This is called a thyroglossal cyst.
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396
How does a brachial cyst arise?
A branchial cyst is a congenital abnormality arising when the second branchial cleft fails to properly form during fetal development. This leaves a space surrounded by epithelial tissue in the lateral aspect of the neck. This space can fill with fluid. This fluid filled lump is called a branchial cyst. Branchial cysts arising from the first, third and fourth branchial clefts are possible, although they are much more rare.
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397
What is transient synovitis?
Also called irritable hip
Temporary irritation and inflammation in the synovial membrane of a joint
Associated with virtual URTI
398
Most common bacteria in osteomyelitis?
Staph aureus
399
There is often a risk factor that predisposes the child to developing osteomyelitis:
Watchman, Thomas. Zero to Finals Paediatrics (p. 222). Kindle Edition.
• Open bone fracture • Orthopaedic surgery • Immunocompromised • Sickle cell anaemia • HIV • Tuberculosis
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400
Presentation of osteomyleitis?
• Refusing to use the limb or weight bear • Pain • Swelling • Tenderness
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401
Talipes equinovarus describes
what
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plantar flexion and supination.
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402
Talipes calcaneovalgus describes what
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in dorsiflexion and pronation.
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403
What is ponseti method?
way of treating talipes without surgery. It is usually very successful. Treatment is started almost immediately after birth. It is performed by a properly trained therapist. The foot is manipulated towards a normal position and a cast is applied to hold it in position. This is repeated over and over until the foot is in the correct position. At some point an achilles tenotomy is performed to release tension in the achilles tendon, often in clinic.
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404
WHat is DDH
Developmental dysplasia of the hip (DDH) is a condition where there are structural abnormalities in the hips caused by abnormal development of the fetal bones during pregnancy. This leads to instability in the hips and a tendency or potential for subluxation or dislocation. These structural abnormalities have the potential to persist into adulthood, leading to weakness, recurrent subluxation or dislocation, abnormal gait and early degenerative changes.
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405
Risk factors for DDH
• First degree family history • Breech presentation from 36 weeks onwards • Breech presentation at birth (after 28 weeks gestation) • Multiple pregnancy
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406
Findings that may suggest DDH are:
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• Different leg lengths • Restricted hip abduction on one side • Significant bilateral restriction in abduction • Difference in the knee level when the hips are flexed • Clunking of the hips on special tests
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407
There are two special tests used to check for DDH:
Watchman, Thomas. Zero to Finals Paediatrics (p. 224). Kindle Edition.
• Ortolani test • Barlow test
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408
Treatment of DDH
Pavlik harness if the baby presents at less than 6 months of age. The Pavlik harness is fitted and kept on permanently, adjusting for the growth of the baby. The aim is to hold the femoral head in the correct position to allow the hip socket (acetabulum) to develop a normal shape. This harness keeps the baby’s hips flexed and abducted. The child is regularly reviewed and the harness is removed when their hips are more stable, usually after 6 - 8 weeks. Surgery is required when the harness fails or the diagnosis is made after 6 months of age. After surgery is performed, an hip spica cast is used to immobilises the hip for a prolonged period.
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Osgood-Schlatter disease is caused BY WHAT
Watchman, Thomas. Zero to Finals Paediatrics (p. 228). Kindle Edition.
inflammation at the tibial tuberosity where the patella ligament inserts. It is a common cause of anterior knee pain in adolescents. It typically occurs in patients aged 10 – 15 years, and is more common in males. Osgood-Schlatter disease is usually unilateral, but it can be bilateral.
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410
Pathophysiology of Osgood schlatter disease
The patella tendon inserts into the tibial tuberosity. The tibial tuberosity is at the epiphyseal plate. Stress from running, jumping and other movements at the same time as growth in the epiphyseal plate result in inflammation on the tibial epiphyseal plate. There are multiple small avulsion fractures, where the patella ligament pulls away tiny pieces of the bone. This leads to growth of the tibial tuberosity, causing a visible lump below the knee. Initially this lump is tender due to the inflammation, but as the bone heals and the inflammation settles it becomes hard and non-tender.
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Osgood-Schlatter disease presents with a gradual onset of symptoms:
Watchman, Thomas. Zero to Finals Paediatrics (p. 229). Kindle Edition.
• Visible or palpable hard and tender lump at the tibial tuberosity • Pain in the anterior aspect of the knee • The pain is exacerbated by physical activity, kneeling and on extension of the knee
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Management of Osgood Schlatter
• Reduction in physical activity • Ice • NSAIDS (ibuprofen) for symptomatic relief
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413
What is osteogenesis imperfecta
Osteogenesis imperfecta is a genetic condition that results in brittle bones that are prone to fractures. It is also knowns as brittle bone syndrome. It is caused by a range of genetic mutations that affect the formation of collagen.
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414
What is collagen
Collagen is a protein that is essential in maintaining the structure and function of bone, as well as the skin, tendons and other connective tissues. There are 8 types of osteogenesis imperfecta depending on the underlying genetic mutation, and they vary in their severity.
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Presentation of osteogenesis imperfecta?
• Hypermobility • Blue / grey sclera (the “whites” of the eyes) • Triangular face • Short stature • Deafness from early adulthood • Dental problems, particularly with formation of teeth • Bone deformities, such as bowed legs and scoliosis • Joint and bone pain
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416
Management of osteogenesis imperfecta
clinical diagnosis. Xrays can be helpful in diagnosing fractures and bone deformities. Genetic testing is possible but not always done routinely.
The underlying genetic condition cannot be cured. Medical treatments include: • Bisphosphates to increase bone density • Vitamin D supplementation to prevent deficiency
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417
What is systemic JIA also called?
Still's disease
418
Features of systemic JIA
• Subtle salmon-pink rash • High swinging fevers • Enlarged lymph nodes • Weight loss • Joint inflammation and pain • Splenomegaly • Muscle pain • Pleuritis and pericarditis
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419
Markers in systemic JIA
Antinuclear antibodies and rheumatoid factor are typically negative. There will be raised inflammatory markers, with raised CRP, ESR, platelets and serum ferritin.
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What is a key complication of systemic JIA
A key complication is macrophage activation syndrome (MAS), where there is severe activation of the immune system with a massive inflammatory response. It presents with an acutely unwell child with disseminated intravascular coagulation (DIC), anaemia, thrombocytopenia, bleeding and a non-blanching rash. It is life threatening. A key investigation finding is a low ESR.
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What is polyarticular JIA
idiopathic inflammatory arthritis in 5 joints or more. The inflammatory arthritis tends to be symmetrical and can affect the small joints of the hands and feet, as well as the large joints such as the hips and knees. There are minimal systemic symptoms, but there can be mild fever, anaemia and reduced growth. Systemic symptoms are mild, unlike systemic onset JIA.
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422
What is oligoartciular JIA also known as
Pauciarticular JIA
423
What is pauciaerticular JIA
4 joints or less
Usually it only affects a single joint, which is described as a monoarthritis. It tends to affect the larger joints, often the knee or ankle. It occurs more frequently in girls under the age of 6 years.
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424
A classic condition associated with oligoarticular JIA is what
Watchman, Thomas. Zero to Finals Paediatrics (p. 233). Kindle Edition.
anterior uveitis. Patients should be referred to an ophthalmologist for management and follow up of uveitis.
Watchman, Thomas. Zero to Finals Paediatrics (p. 233). Kindle Edition.
425
Markers in pauciarticular JIA
Patients tend not to have any systemic symptoms and inflammatory makers will be normal or mildly elevated. Antinuclear antibodies are often positive, however rheumatoid factor is usually negative.
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426
What can be thought of as the paediatric version of the seronegative spondyloarthropathy
Watchman, Thomas. Zero to Finals Paediatrics (p. 233). Kindle Edition.
Enthesitis related arthrtitis
427
What is an enthesis?
point at which the tendon inserts into bone.
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428
What is enthesitis?
Inflammation of the insertion point
can be caused by traumatic stress, such as through repetitive strain during sporting activities, or can be caused by an autoimmune inflammatory process.
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majority of patients with enthesitis-related arthritis have what gene?
Watchman, Thomas. Zero to Finals Paediatrics (p. 233). Kindle Edition.
HLA B27 gene
430
What is psoriatic arthritis?
seronegative inflammatory arthritis associated with psoriasis, the skin condition. The pattern of joint involvement varies. Patients can have a symmetrical polyarthritis affecting the small joints, similar to rheumatoid arthritis, or an asymmetrical arthritis affecting the large joints in the lower limb.
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431
Juvenile psoriatic arthritis is associated with several signs on examination:
Watchman, Thomas. Zero to Finals Paediatrics (p. 234). Kindle Edition.
• Plaques of psoriasis on the skin • Pitting of the nails (nail pitting) • Onycholysis, separation of the nail from the nail bed • Dactylitis, inflammation of the full finger • Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone
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432
Medical treatment of JIA
• NSAIDs, such as ibuprofen • Steroids (oral, intramuscular or intra-artricular) • Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide • Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab
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433
What is ehler's danlos syndrme
umbrella term that encompasses a group of genetic conditions that cause defects in collagen, resulting in hypermobility of the patient’s joints and abnormalities in connective tissue such as the skin, bones, blood vessels and organs. There are several types of Ehlers-Danlos syndrome. This section mainly focuses on hypermobile Ehlers-Danlos syndrome.
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434
Hypermobile Ehlers-Danlos syndrome represents what
Watchman, Thomas. Zero to Finals Paediatrics (p. 234). Kindle Edition.
most cases of EDS in clinical practice and exams. It is the most common and least severe type of Ehlers-Danlos syndrome. The key feature is joint hypermobility, but patients also have soft and stretchy skin. The gene for hypermobile EDS has not been identified and there is no single mode of inheritance.
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435
What is classical ehlers danlos syndrome
features remarkably stretchy skin that feels smooth and velvety to touch. They also have severe joint hypermobility, joint pain and abnormal wound healing. They can develop lumps over pressure points, such as the elbows. They are prone to hernias, prolapses,mitral regurgitation and aortic root dilatation. Inheritance is autosomal dominant.
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436
What is vascular ehlers danlos syndrome
most dangerous form of EDS. The blood vessels are particularly fragile as a result of defective collagen. Patients have characterise thin, translucent skin that you can almost see through. The skin, internal organs and arteries are fragile and prone to rupturing. Patients are monitored for vascular abnormalities and told to seek urgent medical attention for sudden unexplained pain or bleeding. Inheritance is autosomal dominant.
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437
What is kyphoscoliotic ehlers danlos syndrome
characterised initially by poor tone (hypotonia) as a neonate and infant, followed by kyphoscoliosis as they grow. There is significant joint hypermobility. Patients tend to be tall and slim. There is a risk of rupture in the medium sized arteries. Inheritance is autosomal dominant.
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438
The most common presenting complaint for hypermobile EDS is what
Watchman, Thomas. Zero to Finals Paediatrics (p. 235). Kindle Edition.
joint pain and hypermobility, however abnormalities in collagen make it a multi-system disorder that leads to symptoms across multiple areas of the body
439
The Beighton score is used to assess what
Watchman, Thomas. Zero to Finals Paediatrics (p. 235). Kindle Edition.
extent of hypermobility and support the diagnosis of hypermobility syndrome. One point is scored for each side of the body, with a maximum score of 9: • Palms flat on the floor with straight legs (scores 1) • Elbows hyperextend • Knees hyperextend • Thumb can bend to touch the forearm • Little finger hyperextends past 90 degrees
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440
Postural orthostatic tachycardia syndrome (POTS) can occur with what
Watchman, Thomas. Zero to Finals Paediatrics (p. 235). Kindle Edition.
can occur with hypermobile Erlers-Danlos syndrome. It is a result of autonomic dysfunction. This causes inappropriate tachycardia on sitting or standing up, resulting in distressing symptoms such as presyncope, syncope, headaches, disorientation, nausea and tremor.
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441
What is Henoch-Schonlein Purpura (HSP)
Watchman, Thomas. Zero to Finals Paediatrics (p. 236). Kindle Edition.
IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children. Inflammation occurs in the affected organs due to IgA deposits in the blood vessels. It affects the skin, kidneys and gastro-intestinal tract. The condition is often triggered by an upper airway infection or gastroenteritis. It is most common in children under the age of 10 years.
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442
The four classic features of henoch schonlein purpura
Watchman, Thomas. Zero to Finals Paediatrics (p. 236). Kindle Edition.
• Purpura (100%), • Joint pain (75%), • Abdominal pain (50%) • Renal involvement (50%)
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443
What is purpura
red-purple lumps under the skin, containing blood.
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444
Kawasaki disease is also known as
Watchman, Thomas. Zero to Finals Paediatrics (p. 237). Kindle Edition.
mucocutaneous lymph node syndrome.
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What is Kawasaki disease?
a systemic, medium-sized vessel vasculitis. It affects young children, typically under 5 years. There is no clear cause or trigger. It is more common in Asian children, particularly Japanese and Korean children. It is also more common in boys. A key complication is coronary artery aneurysm.
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Clinical features of kawasaki disease?
A key feature that should make you consider Kawasaki disease is a persistent high fever (above 39ºC) for more than 5 days. Children will be unhappy and unwell. The key skin findings are a widespread erythematous maculopapular rash and desquamation (skin peeling) on the palms and soles. Other features include: • Strawberry tongue (red tongue with large papillae) • Cracked lips • Cervical lymphadenopathy • Bilateral conjunctivitis
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447
There are three phases to Kawasaki disease:
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• Acute phase: The child is most unwell with the fever, rash and lymphadenopathy. This lasts 1 - 2 weeks. • Subacute phase: The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 - 4 weeks. • Convalescent stage: The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 - 4 weeks.
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448
There are two first line medical treatments given to patients with Kawasaki disease:
Watchman, Thomas. Zero to Finals Paediatrics (p. 238). Kindle Edition.
• High dose aspirin to reduce the risk of thrombosis • IV immunoglobulins to reduce the risk of coronary artery aneurysms
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449
What is rheumatic fever
an autoimmune condition triggered by streptococcus bacteria. It is caused by antibodies created against the streptococcus bacteria that also target tissues in the body. It is a multi-system disorder that affects the joints, heart, skin and nervous system. It is rare in the UK due to early treatment of streptococcus with antibiotics.
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450
Pathophysiology of rheumatic fever
caused by group A beta-haemolytic streptococci, typically streptococcus pyogenes causing tonsillitis. The immune system creates antibodies to fight the infection. These antibodies not only target the bacteria, but also match antigens on the cells of the person’s body, for example the muscle cells in the myocardium in the heart. This results in a type 2 hypersensitivity reaction, where the immune system begins attacking cells throughout the body. This process is usually delayed 2 - 4 weeks after the initial infection.
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451
The typical presentation of rheumatic fever occurs
Watchman, Thomas. Zero to Finals Paediatrics (p. 239). Kindle Edition.
occurs 2 - 4 weeks following a streptococcal infection, such as tonsillitis.
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452
What does rheymatic fever cause?
migratory arthritis affecting the large joints. It causes hot, swollen and painful joints. It is migratory because different joints become inflamed and improve at different times, giving the appearance that the arthritis is moving from one joint to the next.
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453
There are two key skin findings with rheumatic fever:
Watchman, Thomas. Zero to Finals Paediatrics (p. 239). Kindle Edition.
• Subcutaneous nodules • Erythema marginatum rash
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454
The erythema marginatum rash involves what
Watchman, Thomas. Zero to Finals Paediatrics (p. 239). Kindle Edition.
pink rings of varying sizes affecting the torso and proximal limbs.
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455
A diagnosis of rheumatic fever can be made when there is how much fo the Jones criteria
Watchman, Thomas. Zero to Finals Paediatrics (p. 240). Kindle Edition.
is evidence of recent streptococcal infection, plus: • Two major criteria OR • One major criteria plus two minor criteria
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456
The mnemonic for the Jones criteria is JONES - FEAR.
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• J - Joint arthritis • O - Organ inflammation, such as carditis • N - Nodules • E - Erythema marginatum rash • S - Sydenham chorea Minor Criteria: • Fever • ECG changes (prolonged PR interval) without carditis • Arthralgia without arthritis • Raised inflammatory markers (CRP and ESR)
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457
Tonsillitis caused by streptococcus should be treated with what
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with phenoxymethylpenicillin (penicillin V) for 10 days.
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458
Patients with clinical features of rheumatic fever should be referred immediately for specialist management. Management involves medications and follow up:
Watchman, Thomas. Zero to Finals Paediatrics (p. 240). Kindle Edition.
* NSAIDs (e.g. ibuprofen) are helpful for treating joint pain • Aspirin and steroids are used to treat carditis • Prophylactic antibiotics (oral or intramuscular penicillin) are used to prevent further streptococcal infections and recurrence of the rheumatic fever. These are continued into adulthood.
* Monitoring and management of complications
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Complications of rheumatic fever
• Recurrence of the rheumatic fever • Valvular heart disease, most notably mitral stenosis • Chronic heart failure
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460
What is eczema
chronic atopic condition caused by defects in the normal continuity of the skin barrier, leading to inflammation in the skin.
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461
The simplified pathophysiology is that eczema is caused by
Watchman, Thomas. Zero to Finals Paediatrics (p. 242). Kindle Edition.
defects in the barrier that the skin provides. Tiny gaps in the skin barrier provide an entrance for irritants, microbes and allergens. These entrants stimulate an immune response, resulting in inflammation and the associated symptoms.
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462
What can management of asthma be split into?
Maintainence and flares
463
What is the key to maintainence
create an artificial barrier over the skin to compensate for the defective skin barrier. This is done using emollients that are as thick and greasy as tolerated, used as often as possible, particularly after washing and before bed. Patients should avoid activities that break down the skin barrier, such as bathing in hot water, scratching or scrubbing their skin and using soaps and body washes that remove the natural oils in the skin. Emollients or specifically designed soap substitutes can be used instead of soap and body washes when showering or washing hands. Some patients find certain environmental factors play a role in making their eczema symptoms worse or better. For example, the eczema may completely resolve on holiday in warm, humid countries, only to flare on returning to the cold air in the UK. Environmental triggers, such as changes in temperature, certain dietary products, washing powders, cleaning products and emotional events or stresses can also play a role.
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What can flares be treated with?
can be treated with thicker emollients, topical steroids, “wet wraps” (covering affected areas in a thick emollient and applying a wrap to keep moisture locked in overnight) and treating any complications such as bacterial or viral infections. Very rarely IV antibiotics or oral steroids might be required in very severe flares.
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465
Thin creams examples
• E45 • Diprobase cream • Oilatum cream • Aveeno cream • Cetraben cream • Epaderm cream
Watchman, Thomas. Zero to Finals Paediatrics (p. 243). Kindle Edition.
466
Examples of thick greasy emollients
• 50:50 ointment (50% liquid paraffin) • Hydromol ointment • Diprobase ointment • Cetraben ointment • Epaderm ointment
Watchman, Thomas. Zero to Finals Paediatrics (p. 243). Kindle Edition.
467
The steroid ladder from weakest to most potent:
Watchman, Thomas. Zero to Finals Paediatrics (p. 243). Kindle Edition.
• Mild: Hydrocortisone 0.5%, 1% and 2.5% • Moderate: Eumovate (clobetasone butyrate 0.05%) • Potent: Betnovate (betamethasone 0.1%) • Very potent: Dermovate (clobetasol propionate 0.05%)
Watchman, Thomas. Zero to Finals Paediatrics (p. 243). Kindle Edition.
468
What is a bacterial infection for eczema
Opportunistic bacterial infection of the skin is common in eczema. The breakdown in the skin’s protective barrier allows an entry point for infective organisms. The most common organism is staphylococcus aureus. Treatment is with oral antibiotics, particularly flucloxacillin. More severe cases may require admission and intravenous antibiotics.
Watchman, Thomas. Zero to Finals Paediatrics (p. 243). Kindle Edition.
469
What is eczema herpeticum
a viral skin infection in patients with eczema caused by the herpes simplex virus (HSV) or varicella zoster virus (VZV). Patients can be very unwell.
Watchman, Thomas. Zero to Finals Paediatrics (p. 243). Kindle Edition.
470
Presentation of eczema herpeticum
typical presentation is a patient who suffers with eczema that has developed a widespread, painful, vesicular rash with systemic symptoms such as fever, lethargy, irritability and reduced oral intake. There will usually be lymphadenopathy (swollen lymph nodes).
Watchman, Thomas. Zero to Finals Paediatrics (p. 244). Kindle Edition.
471
What does the rash look like in eczema herpeticum
The rash is usually widespread and can affect any area of the body. It is erythematous, painful and sometimes itchy, with vesicles containing pus. The vesicles appear as lots of individual spots containing
fluid. After they burst, they leave small punched-out ulcers with a red base.
Watchman, Thomas. Zero to Finals Paediatrics (p. 244). Kindle Edition.
472
Management of eczema herpeticum
Viral swabs of the vesicles can be used to confirm the diagnosis, although treatment is usually started based on the clinical appearance. Treatment is with aciclovir. A mild or moderate case may be treated with oral aciclovir, whereas more severe cases may require IV aciclovir.
Watchman, Thomas. Zero to Finals Paediatrics (p. 244). Kindle Edition.
473
What is psoriasis
chronic autoimmune condition that causes recurrent symptoms of psoriatic skin lesions. There is a large variation in how severely patients are affected with psoriasis. There appears to be a genetic component but no clear genetic inheritance has been established. Around a third of patients have a first degree relative with psoriasis. The symptoms start in childhood in a third of patients.
Watchman, Thomas. Zero to Finals Paediatrics (p. 244). Kindle Edition.
474
What is plaque psoriasis
psoriasis features thickened erythematous plaques with silver scales, commonly seen on the extensor surfaces and scalp. The plaques are 1cm - 10cm in diameter. This is the most common form of psoriasis in adults.
Watchman, Thomas. Zero to Finals Paediatrics (p. 244). Kindle Edition.
475
What is guttate psoriasis?
second most common form of psoriasis and commonly occurs in children. It presents with many small raised papules across the trunk and limbs. The papules are mildly erythematous and can be slightly scaly. Over time the papules in guttate psoriasis can turn into plaques. Guttate psoriasis is often triggered by a streptococcal throat infection, stress or medications. It often resolves spontaneously within 3 - 4 months.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 244-245). Kindle Edition.
476
What is pustular psoriasis
rare severe form of psoriasis where pustules form under areas of erythematous skin. The pus in these areas is not infectious. Patients can be systemically unwell. It should be treated as a medical emergency and patients with pustular psoriasis initially require admission to hospital.
Watchman, Thomas. Zero to Finals Paediatrics (p. 245). Kindle Edition.
477
What is erythrodermic psoriasis?
rare severe form of psoriasis with extensive erythematous inflamed areas covering most of the surface area of the skin. The skin comes away in large patches (exfoliation) resulting in raw exposed areas. It should be treated as a medical emergency and patients require admission.
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478
There are a few specific signs suggestive of psoriasis:
Watchman, Thomas. Zero to Finals Paediatrics (p. 245). Kindle Edition.
• Auspitz sign refers to small points of bleeding when plaques are scraped off • Koebner phenomenon refers to the development of psoriatic lesions in areas of skin affected by trauma • Residual pigmentation of the skin after the lesions resolve
Watchman, Thomas. Zero to Finals Paediatrics (p. 245). Kindle Edition.
479
Treatment options for psoariasis
• Topical steroids • Topical vitamin D analogues (calcipotriol) • Topical dithranol • Topical calcineurin inhibitors (tacrolimus) are usually only used in adults • Phototherapy with narrow band ultraviolet B light is particularly useful in extensive guttate psoriasis
Watchman, Thomas. Zero to Finals Paediatrics (p. 245). Kindle Edition.
480
Psoriasis associations
Nail psoriasis describes the nail changes that can occur in patients with psoriasis. These include nail pitting, thickening, discolouration, ridging and onycholysis (separation of the nail from the nail bed).
Psoriatic arthritis occurs in 10 - 20% of patients with psoriasis and usually occurs within 10 years of developing the skin changes. It typically affects people in middle age but can occur at any age. There are psychosocial implications of having chronic skin lesions, which may affect mood, self esteem and social acceptance and cause depression and anxiety.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 245-246). Kindle Edition.
481
What is acne vulgaris
extremely common condition, often affecting people during puberty and adolescence. Most people are affected at some point during their lives, and symptoms can range from mild to severe.
Watchman, Thomas. Zero to Finals Paediatrics (p. 246). Kindle Edition.
482
What is acne caused by?
chronic inflammation, with or without localised infection, in pockets within the skin known as the pilosebaceous unit. The pilosebaceous units are the tiny dimples in the skin that contain the hair follicles and sebaceous glands. The sebaceous glands produce the natural skin oils and a waxy substance known as sebum.
Watchman, Thomas. Zero to Finals Paediatrics (p. 246). Kindle Edition.
483
What does acne result from?
increased production of sebum, trapping of keratin (dead skin cells) and blockage of the pilosebaceous unit. This leads to swelling and inflammation in the pilosebaceous unit. Androgenic hormones increase the production of sebum, which is why acne is exacerbated by puberty and improves with anti-androgenic hormonal contraception. Swollen and inflamed units are called comedones.
Watchman, Thomas. Zero to Finals Paediatrics (p. 246). Kindle Edition.
484
What is propinibacterium acnes role in acne
bacteria is felt to play an important role in acne. This is a bacteria that colonises the skin. It is thought that excessive growth of this bacteria can exacerbate acne.
Watchman, Thomas. Zero to Finals Paediatrics (p. 246). Kindle Edition.
485
Management of acne
• No treatment may be acceptable if mild • Topical benzoyl peroxide reduces inflammation, helps unblock the skin and is toxic to the P. acnes bacteria
• Topical retinoids (chemicals related to vitamin A) slow the production of sebum • Topical antibiotics such as clindamycin (co-prescribed with benzoyl peroxide to reduce bacterial resistance) • Oral antibiotics such as lymecycline • Oral contraceptive pill can help female patients stabilise their hormones and slow the production of sebum
Oral retinoids for severe acne (i.e. isotretinoin) is an effective last-line option, although it is only prescribed by a specialist after other methods fail. This needs careful follow up and monitoring and reliable contraception in females.
Co-cyprindiol (Dianette) is the most effective combined contraceptive pill for acne due to it’s anti-androgen effects. It has a higher risk of thromboembolism, so treatment is usually discontinued once acne is controlled and it is not prescribed long term.
Watchman, Thomas. Zero to Finals Paediatrics (p. 247). Kindle Edition.
486
Side effects of isotretinoin include:
Watchman, Thomas. Zero to Finals Paediatrics (p. 247). Kindle Edition.
• Dry skin and lips • Photosensitivity of the skin to sunlight • Depression, anxiety, aggression and suicidal ideation. Patients should be screened for mental health issues prior to starting treatment. • Rarely Stevens-Johnson syndrome and toxic epidermal necrolysis
Watchman, Thomas. Zero to Finals Paediatrics (p. 247). Kindle Edition.
487
What is measles caused by
Measles virus
488
How. ismeasles transmitted
Respiratoru droplets
489
Symtpoms of measles
10-12 days after exposure with fever, coryzal and conjunctivitis
490
What are koplik spots?
are greyish white spots on the buccal mucosa. They appear 2 days after the fever. They are pathognomonic for measles, meaning if a patient has Koplik spots, you can diagnose measles.
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
491
Complications of measles
• Pneumonia • Diarrhoea • Dehydration • Encephalitis • Meningitis • Hearing loss • Vision loss • Death
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
492
What is scarlet fever associated with
group A streptococcus infection, usually tonsillitis. It is not caused by a virus.
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
493
Scarlet fever is caused by what
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
exotoxin produced by the streptococcus pyogenes (group A strep) bacteria. It is characterised by a red-pink, blotchy, macular rash with rough “sandpaper” skin that starts on the trunk and spreads outwards. Patients can have red, flushed cheeks.
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
494
Scarlet fever treatment
antibiotics for the underlying streptococcal bacterial infection. This is with phenoxymethylpenicillin (penicillin V) for 10 days. Scarlet fever is a notifiable disease and all cases need to be reported to public health. Children should be kept off school until 24 hours after starting antibiotics.
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
495
What is rubella caused by?
the rubella virus. It is highly contagious and spread by respiratory droplets. Symptoms start 2 weeks after exposure.
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
496
What does rubella present with
milder erythematous macular rash compared with measles. The rash starts on the face and spreads to the rest of the body. The rash classically lasts 3 days. It can be associated with a mild fever, joint pain and a sore throat. Patients often have enlarged lymph nodes (lymphadenopathy) behind the ears and at the back of the neck.
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
497
Rubella management
is supportive and the condition is self limiting. Rubella is a notifiable disease and all cases need to be reported to public health. Children should stay off school for at least 5 days after the rash appears. Children should avoid pregnant women.
Watchman, Thomas. Zero to Finals Paediatrics (p. 248). Kindle Edition.
498
Complications of rubella
thrombocytopenia and encephalitis. Rubella is dangerous in pregnancy and can lead to congenital rubella syndrome, which is a triad of deafness, blindness and congenital heart disease.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 248-249). Kindle Edition.
499
What does slapped cheek start presenting as
mild fever, coryza and non-specific viral symptoms such as muscle aches and lethargy. After 2 - 5 days the rash appears quite rapidly as a diffuse bright red rash on both cheeks, as though they have “slapped cheeks”. A few days later a reticular mildly erythematous rash affecting the trunk and limbs appears that can be raised and itchy. Reticular means net-like.
Watchman, Thomas. Zero to Finals Paediatrics (p. 249). Kindle Edition.
500
Complications of slapped cheek
• Aplastic anaemia • Encephalitis or meningitis • Pregnancy complications including fetal death • Rarely hepatitis, myocarditis or nephritis
Watchman, Thomas. Zero to Finals Paediatrics (p. 249). Kindle Edition.
501
What is roseola infantum caused by?
caused by human herpesvirus 6 (HHV-6) and less frequently by human herpesvirus 7 (HHV-7).
Watchman, Thomas. Zero to Finals Paediatrics (p. 249). Kindle Edition.
502
Roseola pattern of illness
presents 1 - 2 weeks after infection with a high fever (up to 40ºC) that comes on suddenly, lasts for 3 - 5 days and then disappears suddenly. There may be coryzal symptoms, sore throat and swollen lymph nodes during the illness. When the fever settles, the rash appears for 1 - 2 days. The rash consists of a mild erythematous macular rash across the arms, legs, trunk and face and is not itchy.
Watchman, Thomas. Zero to Finals Paediatrics (p. 249). Kindle Edition.
503
Main complication of roseola infantum?
febrile convulsions due to high temperatures. Immunocompromised patients may be at risk of rare complications such as myocarditis, thrombocytopenia and Guillain-Barre syndrome.
Watchman, Thomas. Zero to Finals Paediatrics (p. 249). Kindle Edition.
504
What is erythema multiforme
Ertyhmatous rash caused by a hypersensitivity reaction
505
What is the presentation of erythema multiforme?
widespread, itchy, erythematous rash. It produces characteristic “target lesions”. Target lesions are red rings within larger red rings, with the darkest red at the centre, similar to a bulls-eye target. It does not usually affect the mucous membranes but can cause a sore mouth (stomatitis). The symptoms come on abruptly over a few days. It may be associated with other symptoms of mild fever, stomatitis, muscle and joint aches, headaches and general flu-like symptoms.
Watchman, Thomas. Zero to Finals Paediatrics (p. 250). Kindle Edition.
506
What is urticaria caused by?
release of histamine and other pro-inflammatory chemicals by mast cells in the skin. This may be part of an allergic reaction in acute urticaria or an autoimmune reaction in chronic idiopathic urticaria.
Watchman, Thomas. Zero to Finals Paediatrics (p. 250). Kindle Edition.
507
Acute urticaria is triggered by something that stimulates the mast cells to release histamine. This may be:
Watchman, Thomas. Zero to Finals Paediatrics (p. 251). Kindle Edition.
• Allergies to food, medications or animals • Contact with chemicals, latex or stinging nettles • Medications • Viral infections • Insect bites • Dermatographism (rubbing of the skin)
Watchman, Thomas. Zero to Finals Paediatrics (p. 251). Kindle Edition.
508
Management of urticuaria
Antihistamines are the main treatment for urticaria. Fexofenadine is usually the antihistamine of choice for chronic urticaria. A short course of oral steroids may be considered for severe flares.
Watchman, Thomas. Zero to Finals Paediatrics (p. 251). Kindle Edition.
509
What is chickenpox caused by
caused by the varicella zoster virus (VZV).
Watchman, Thomas. Zero to Finals Paediatrics (p. 251). Kindle Edition.
510
What does chicken pox present like
Chickenpox is characterised by widespread, erythematous, raised, vesicular (fluid filled), blistering lesions. The rash usually starts on the trunk or face and spreads outwards affecting the whole body over 2 - 5 days. Eventually the lesions scab over, at which point they stop being contagious. Other symptoms: • Fever is often the first symptom • Itch • General fatigue and malaise
Watchman, Thomas. Zero to Finals Paediatrics (pp. 251-252). Kindle Edition.
511
When do patients stop being infectious with chicken pox
become symptomatic 10 days to 3 weeks after exposure. They stop being contagious after all the lesions have crusted over.
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512
Complications of chicken pox
• Bacterial superinfection • Dehydration • Conjunctival lesions • Pneumonia • Encephalitis (presenting as ataxia) After the infection the virus can lie dormant in the sensory dorsal root ganglion cells and cranial nerves, and reactivate later in life as shingles or Ramsay Hunt syndrome.
Watchman, Thomas. Zero to Finals Paediatrics (p. 252). Kindle Edition.
513
WHat is hand foot and mouth disease caused by
the coxsackie A virus.
Watchman, Thomas. Zero to Finals Paediatrics (p. 253). Kindle Edition.
514
Presentation of hand foot and mouth
The illness starts with typical viral upper respiratory tract symptoms such as tiredness, sore throat, dry cough and raised temperature. After 1 - 2 days small mouth ulcers appear, followed by blistering red spots across the body. As the name suggests, these spots are most notable on the hands, feet and around the mouth. Painful mouth ulcers, particularly on the tongue are also a key feature. The rash may be itchy.
Watchman, Thomas. Zero to Finals Paediatrics (p. 253). Kindle Edition.
515
Management of hand foot and mouth disease
There is no treatment for hand, foot and mouth disease. Management is supportive, with adequate fluid intake and simple analgesia such as paracetamol if required. The rash and illness resolve spontaneously without treatment after a week to 10 days
It is highly contagious and advice should be given about measures to avoid transmission, such as avoiding sharing towels and bedding, washing hands and careful handling of dirty nappies.
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516
What is mollucsum contagiosum
viral skin infection caused by the molluscum contagiosum virus, which is a type of poxvirus.
Watchman, Thomas. Zero to Finals Paediatrics (p. 253). Kindle Edition.
517
What is mollucsum contagiousum characterised by
small, flesh coloured papules (raised individual bumps on the skin) that characteristically have a central dimple. They appear in “crops” of multiple lesions in a local area. It is spread through direct contact or by sharing items such as towels or bedsheets. The papules resolve spontaneously without any treatment, however this can take up to 18 months. Once they resolve the skin returns to normal. Scratching or picking the lesions should be avoided as it can lead to spreading, scarring and infection.
Watchman, Thomas. Zero to Finals Paediatrics (p. 253). Kindle Edition.
518
Management of mollucsum contagiosum
No treatment or change in lifestyle is required and children can continue all their normal activities. They should avoid sharing towels or close contact with the lesions to minimise the risk of spreading the infection. Usually reassurance and education is all that is required.
Rarely, if bacterial superinfection infection occurs in the lesions as a result of scratching, this may require treatment with antibiotics. Options include topical fusidic acid or oral flucloxacillin.
Watchman, Thomas. Zero to Finals Paediatrics (p. 254). Kindle Edition.
Watchman, Thomas. Zero to Finals Paediatrics (p. 254). Kindle Edition.
519
What is pityriasis rosea?
generalised, self limiting rash that has an unknown cause. It typically occurs in adolescents and young adults. It may be caused by a virus such as the human herpes virus (HHV-6 or HHV-7), but no definitive causative organism had been established.
Watchman, Thomas. Zero to Finals Paediatrics (p. 254). Kindle Edition.
520
Presentation of pituriasis rosea?
There may be prodromal symptoms prior to the rash developing. These include headache, tiredness, loss of appetite and flu-like symptoms. The rash starts with a characteristic herald patch. This is a faint red or pink, scaly, oval shaped lesion that is 2cm or more in diameter, usually occurring somewhere on the torso. It appears 2 or more days prior to the rest of the rash.
The rash consists of widespread faint red or pink, slightly scaly, oval shaped lesions, usually less than 2 cm in diameter. On the torso they can be arranged in a characteristic “christmas tree” fashion, following the lines of the ribs. In darker skinned patients the lesions can be grey coloured, lighter or darker than their skin colour.
Watchman, Thomas. Zero to Finals Paediatrics (p. 254). Kindle Edition.
521
Disease course of pityriasis rosea?
The rash resolves without treatment within 3 months. It can leave a discolouration of the skin where the lesions were, however these will also resolved within another few months.
Watchman, Thomas. Zero to Finals Paediatrics (p. 254). Kindle Edition.
522
What is seborrheic dermtitisi
inflammatory skin condition that affects the sebaceous glands. The sebaceous glands are the oil producing glands in the skin. It affects areas that have a lot of these glands, such as the scalp, nasolabial folds and eyebrows.
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
523
Infantile seborrhoeic dermatitis (cradle cap) causes what
crusted flaky scalp. It is a self limiting condition and usually resolves by 4 months of age, but can last until 12 months.
524
Management of infantile sebrorheic dermatitis?
First line treatment is by applying baby oil, vegetable oil or olive oil, gently brushing the scalp then washing off. When this is not effective, white petroleum jelly can be used overnight to soften the crusted areas before washing off in the morning.
The next step is a topical anti-fungal cream such as clotrimazole or miconazole, used for up to 4 weeks. Severe or unresponsive cases may need referral to a dermatologist.
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
525
Mild seborrhoeic dermatitis of the scalp presents with what
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
flaky itchy skin on the scalp (dandruff). More severe cases cause dense oily scaly brown crusting. This commonly occurs in adolescents and adults rather than children.
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
526
Treatment of mild seborrhoeic dermatitis?
ketoconazole shampoo, left on for 5 minutes before washing off. Topical steroids may be used if there is severe itching. It often reoccurs after successful treatment.
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
527
Seborrhoeic dermatitis of the face and body presents
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
with red, flaky, crusted, itchy skin. It commonly affects the eyelids, nasolabial folds, ears, upper chest and back.
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
528
Management of seborrhoeic dermatitis of the face and body
First line treatment is with an antifungal cream, such as clotrimazole or miconazole, used for up to 4 weeks. Localised inflamed areas may benefit from topical steroids, such as hydrocortisone 1%. Severe or unresponsive cases should be referred to a dermatologist or paediatrician.
Watchman, Thomas. Zero to Finals Paediatrics (p. 255). Kindle Edition.
529
What is ringworm
fungal infection of the skin. It is also known as tinea and dermatophytosis.
Watchman, Thomas. Zero to Finals Paediatrics (p. 256). Kindle Edition.
530
How does ringworm present
presents as an itchy rash that is erythematous, scaly and well demarcated. There is often one or several rings or circular shaped areas that spread outwards, with a well demarcated edge. The edge is more prominent and red and the area in the centre is more faint in colour.
Watchman, Thomas. Zero to Finals Paediatrics (p. 256). Kindle Edition.
531
What is nappy rash
contact dermatitis in the nappy area. It is caused by friction between the skin and nappy, and contact with urine and faeces in a dirty nappy. Most babies will get nappy rash at some point, and it is most common between 9 and 12 months of age. Additionally, the break down in skin and the warm moist environment in the nappy can lead to added infection with fungus (candida) or bacteria, usually staphylococcus or streptococcus.
Watchman, Thomas. Zero to Finals Paediatrics (p. 257). Kindle Edition.
532
Risk factors for nappy rash
• Delayed changing of nappies • Irritant soap products and vigorous cleaning • Certain types of nappies (poorly absorbent ones) • Diarrhoea • Oral antibiotics predispose to candida infection • Pre-term infants
Watchman, Thomas. Zero to Finals Paediatrics (p. 257). Kindle Edition.
533
Presentation of nappy rash
Nappy rash present with sore, red, inflamed skin in the nappy area. The rash appears in individual patches on exposed areas of the skin that come in contact with the nappy. It tends to spare the skin creases, meaning the creases in the groin are healthy. There may be a few red papules beside the affected areas of skin. Nappy rash is uncomfortable, may be itchy and the infant may be distressed.
Watchman, Thomas. Zero to Finals Paediatrics (p. 257). Kindle Edition.
534
Management of nappy rash
• Switching to highly absorbent nappies (disposable gel matrix nappies) • Change the nappy and clean the skin as soon as possible after wetting or soiling • Use water or gentle alcohol free products for cleaning the nappy area • Ensure the nappy area is dry before replacing the nappy • Maximise time not wearing a nappy
Watchman, Thomas. Zero to Finals Paediatrics (p. 258). Kindle Edition.
535
Complications of nappy rash
• Candida infection • Cellulitis • Jacquet's erosive diaper dermatitis • Perianal pseudoverrucous papules and nodules
Watchman, Thomas. Zero to Finals Paediatrics (p. 258). Kindle Edition.
536
What are scabies
tiny mites called Sarcoptes scabiei that burrow under the skin causing infection and intense itching. They lay eggs in the skin, leading to further infection and symptoms. It can take up to 8 weeks for any symptoms or rash to appear after the initial infestation.
Watchman, Thomas. Zero to Finals Paediatrics (p. 258). Kindle Edition.
537
Scabies presentation?
present with incredibly itchy small red spots, possibly with track marks where the mites have burrowed. The classic location of the rash is between the finger webs, but it can spread to the whole body.
Watchman, Thomas. Zero to Finals Paediatrics (p. 258). Kindle Edition.
538
Management of scabies
Treatment is with permethrin cream. This needs to be applied to the whole body, completely covering skin. It is best to do this when the skin is cool (i.e. not after a bath or shower) so that a layer of cream remains on top of the skin and does not get absorbed.
Oral ivermectin as a single dose that can be repeated a week later is an option for difficult to treat or crusted scabies.
Watchman, Thomas. Zero to Finals Paediatrics (p. 258). Kindle Edition.
539
What is headlice
Pediculus humanus capitis parasites, which cause infestations of the scalp, most commonly in school aged children. Head lice are commonly known as nits, however nits are egg shells that have hatched or contain unviable embryos and not the lice themselves.
Watchman, Thomas. Zero to Finals Paediatrics (p. 259). Kindle Edition.
540
Management of head lice
Dimeticone 4% lotion can be applied to the hair and left to dry. This is left on for 8 hours (i.e. overnight), then washed off. This process is repeated 7 days later to kill any head lice that have hatched since treatment. Special fine combs can be used to systematically comb the nits and lice out of the hair.
Watchman, Thomas. Zero to Finals Paediatrics (p. 259). Kindle Edition.
541
Non-blanching rashes are caused by what
Watchman, Thomas. Zero to Finals Paediatrics (p. 260). Kindle Edition.
bleeding under the skin
542
What are petichae
are small (< 3mm), non-blanching, red spots on the skin caused by burst capillaries.
Watchman, Thomas. Zero to Finals Paediatrics (p. 260). Kindle Edition.
543
What is purpura
larger (3 - 10mm) non-blanching, red-purple, macules or papules created by leaking of blood from vessels under the skin.
Watchman, Thomas. Zero to Finals Paediatrics (p. 260). Kindle Edition.
544
What is the most concerning differential for a non blanching rash?
meningococcal septicaemia. Patients with features of sepsis need immediate management for life threatening meningococcal sepsis.
Watchman, Thomas. Zero to Finals Paediatrics (p. 260). Kindle Edition.
545
What is erythema nodosum
a condition where red lumps appear across the patient’s shins. Erythema means red and nodosum directly translates from Latin as “knots”, referring to lumps.
Watchman, Thomas. Zero to Finals Paediatrics (p. 261). Kindle Edition.
546
What is erythema nodosum caused by/
inflammation of the subcutaneous fat on the shins. Inflammation of fat is called panniculitis. It is caused by a hypersensitivity reaction. In around half of patients there is no identifiable cause. It is associated with a number of triggers and underlying conditions.
Watchman, Thomas. Zero to Finals Paediatrics (p. 261). Kindle Edition.
547
What is erythma nodosum assoiated with
• Streptococcal throat infections • Gastroenteritis • Mycoplasma pneumoniae • Tuberculosis • Pregnancy • Medications, such as the oral contraceptive pill
Watchman, Thomas. Zero to Finals Paediatrics (p. 261). Kindle Edition.
548
Presentation of erythema nodosum?
Erythema nodosum presents with red, inflamed, subcutaneous nodules across both shins. The nodules are raised and can be painful and tender. Over time the nodules settle and appears as bruises. When you suspect someone has erythema nodosum it is important to look for signs and symptoms of potential triggers and underlying medical conditions.
Watchman, Thomas. Zero to Finals Paediatrics (p. 261). Kindle Edition.
549
What is impetigo?
superficial bacterial skin infection, usually caused by the staphylococcus aureus bacteria. A “golden crust” is characteristic of a staphylococcus skin infection. It is also less commonly caused by the streptococcus pyogenes bacteria. Impetigo is contagious and children should be kept off school during the infection. Impetigo occurs when bacteria enter via a break in the skin. This may be in otherwise healthy skin or may be related to eczema or dermatitis.
Watchman, Thomas. Zero to Finals Paediatrics (p. 262). Kindle Edition.
550
How can impetigo be classified?
Non bullous or bullous
551
Where does non bullous impetigo occur
typically occurs around the nose or mouth. The exudate from the lesions dry and form a “golden crust”. They are unsightly but do not usually cause systemic symptoms or make the person unwell.
Watchman, Thomas. Zero to Finals Paediatrics (p. 262). Kindle Edition.
552
Management of non bullous impetigo
Topical fusidic acid can be used to treat localised non-bullous impetigo. Draft NICE guidelines from August 2019 suggest using antiseptic cream (hydrogen peroxide 1% cream) first line rather than antibiotics for localised non-bullous impetigo.
Oral flucloxacillin is used to treat more wide spread or severe impetigo. Flucloxacillin is the antibiotic of choice for staphylococcal infections. Advise about measures to avoid spreading the impetigo. Patients should be given advice about not touching or scratching the lesions, hand hygiene and avoiding sharing face towels and cutlery. They need to be off school until all the lesions have healed or they have been treated with antibiotics for at least 48 hours.
Watchman, Thomas. Zero to Finals Paediatrics (p. 262). Kindle Edition.
553
What is bullous impetigo caused by?
Bullous impetigo is always caused by the staphylococcus aureus bacteria. These bacteria can produce epidermolytic toxins that break down the proteins that hold skin cells together. This causes 1 - 2 cm fluid filled vesicles to form on the skin. These vesicles grow in size and then burst, forming a “golden crust”. Eventually they heal without scarring. These lesions can be painful and itchy.
Watchman, Thomas. Zero to Finals Paediatrics (p. 263). Kindle Edition.
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What is staphylococcal scaled skin syndrome
Staphylococcal scalded skin syndrome (SSSS) is a condition caused by a type of staphylococcus aureus bacteria that produces epidermolytic toxins. These toxins are protease enzymes that break down the proteins that hold skin cells together. When a skin infection occurs and these toxins are produced, the skin is damaged and breaks down. This condition usually affects children under 5 years. Older children and adults have usually developed immunity to the epidermolytic toxins.
Watchman, Thomas. Zero to Finals Paediatrics (p. 263). Kindle Edition.
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What is nikolsky sign?
where very gentle rubbing of the skin causes it to peel away. This is positive in SSSS.
Watchman, Thomas. Zero to Finals Paediatrics (p. 263). Kindle Edition.
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Presentation of staphyloccocal scaled skin syndrome
SSSS usually starts with generalised patches of erythema on the skin. Then the skin looks thin and wrinkled. This is followed by the formation of fluid filled blisters called bullae, which burst and leave very sore, erythematous skin below. This has a similar appearance to a burn or scald.
Watchman, Thomas. Zero to Finals Paediatrics (p. 263). Kindle Edition.
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Management of staohylocccal scaled skin syndrome
Most patients will require admission and treatment with IV antibiotics. Fluid and electrolyte balance is key to management as patients are prone to dehydration. When adequately treated, children usually make a full recovery without scarring.
Watchman, Thomas. Zero to Finals Paediatrics (p. 264). Kindle Edition.
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What are SJS and TEN
spectrum of the same pathology, where a disproportional immune response causes epidermal necrosis, resulting in blistering and shedding of the top layer of skin. Generally, SJS affects less that 10% of the body surface area whereas TEN affects more than 10% of the body surface area. Certain HLA genetic types are at higher risk of SJS and TEN.
Watchman, Thomas. Zero to Finals Paediatrics (p. 264). Kindle Edition.
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Presentation of SJS and TEN
The condition has a spectrum of severity. Some cases are mild whilst others are very severe and can potentially be fatal. Patients usually start with non-specific symptoms of fever, cough, sore throat, sore mouth, sore eyes and itchy skin. They then develop a purple or red rash that spreads across the skin and starts to blister. A few days after the blistering starts, the skin starts to break away and shed leaving the raw tissue underneath. Pain, erythema, blistering and shedding can also happen to the lips and mucous membranes. Eyes can become inflamed and ulcerated. It can also affect the urinary tract, lungs and internal organs.
Watchman, Thomas. Zero to Finals Paediatrics (p. 265). Kindle Edition.
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Management of SJS and TEN
Medical emergencies and patients should be admitted to a suitable dermatology or burns unit for treatment. Good supportive care is essential, including nutritional care, antiseptics, analgesia and ophthalmology input. Treatment options include steroids, immunoglobulins and immunosuppressant medications guided by a specialist.
Watchman, Thomas. Zero to Finals Paediatrics (p. 265). Kindle Edition.
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Complications of SJS and TEN
* Secondary infection: The breaks in the skin can lead to secondary bacterial infection, cellulitis and sepsis. • Permanent skin damage: Skin involvement can lead to scarring and damage to skin, hair, nails, lungs and genitals.
* Visual complications: Depending on the severity, eye involvement can range from sore eyes to severe scarring and blindness.
Watchman, Thomas. Zero to Finals Paediatrics (p. 265). Kindle Edition.
