Zero to final paeds Flashcards
What is bronchioloitis?
Describes inflammation and infection in the bronchioles the small airways of the lungs
What is the most common cause of bronchiolitis?
RSV
What are the sounds heard in lungs on bronchiolitis?
Harsh breath sounds
Wheeze
Crackles
What is the presentation of bronchiolitis?
Coryzal symptoms Signs of respiratory distress Dysponea Tachyponea Poor feeding Mild fever Aponeas Wheeze and crackles
Signs of respiratory distress?
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
What is wheezing?
Whistling sound caused by narrowed airways typically heard during expiration
What is grunting?
Caused by exhaling with the glottis partially closed to increase positive end expiratory pressuer
What is stridor?
High pitched inspiratory noise caused by obstruction of the upper airway for example in croup
What is the typical course of RSV?
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.
Reasons for admission with beonchiolitis?
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
What is the management of bronchiolotisi?
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
What can saline nasal drops and nasal suctioning do?
Clear nasal secretions
What is continous positive airway pressure?
Involves using a sealed nasal cannula
Deliver high and controlled pressure
Signs of poor ventrilation in children?
Raised pCO2
Falling pH
What is palivizumab?
Monoclonal antibody that targets respiratory syncyctial virus
Monthly injection is given as prevention against bronchiolotitis caused by RSV
Given to high risk babies
What is a viral induced wheeze?
acute wheezy illness caused by a viral infection.
Features of VIW
• Presenting before 3 years of age • No atopic history • Only occurs during viral infections
Presentation of VIW?
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.
Presentation of acute asthma?
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.
What does a silent chest suggest?
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.
Staples of management in acute viral induced wheeze or asthma are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
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.
Bronchodilators are stepped up as required:
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
• 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.
How are mild cases of asthma managed?
Salbutamol inhaler via a spacer
Moderate to severe cases require a stepwise approach working upwards until control is achieved:
Watchman, Thomas. Zero to Finals Paediatrics (p. 5). Kindle Edition.
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.
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.
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 otheratopic conditionssuch 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.
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.
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.
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.
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 oralleukotriene receptor antagonist(i.e.montelukast), oraltheophyllineor an inhaledLAMA(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. oralsalbutamol). 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.
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.
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.
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.
What is pneumonia?
Simple infection of lung tissue
Inflammation fo lung tissue and sputum in the airways and alveoli
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.
These can indicatesepsissecondary 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.
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 percussiondue to lung tissue collapse and/or consolidation
Watchman, Thomas. Zero to Finals Paediatrics (p. 10). Kindle Edition.
Most common bacterial cause of pneumonia?
Streptococcus pneumonia
Most common viral cause of pneumonia?
RSV
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.
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.
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.
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.
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.
What is croup?
Acute infective respiratory disease affecting young children
Who does croup typically affect?
Children 6 months to 2 years but can be older
What does croup cause?
Oedema in the larynx
What is the classic cause of croup?
Parainfluenza virus
Common causes of croup?
• Parainfluenza • Influenza • Adenovirus • Respiratory syncytial virus (RSV)
Watchman, Thomas. Zero to Finals Paediatrics (p. 11). Kindle Edition.
Why is croup not caused by diptheria commonly anymore?
Vaccination
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.
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.
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.
What is epiglottitis?
Inflammation and swelling of the epiglottis
Typically with heaemophilus influenza B
Why is epiglottisis now rare?
Due to routine vaccination program
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.
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
What is a common complication of epiglottisi?
Epiglottic abscess
Collection of pus around the epiglottis
Threatens the airways making it a life threateneing emergencu
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.
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.
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.
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.
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
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.
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.
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.
What is a complication of bronchiectasis?
Whooping cough
What is chronic lung disease of prematurity also known as?
Bronchopulmonary dysplasia
Who does chronic lung disease of prematurity occur in?
Premature babies, typically born before 28 weeks gestation
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.
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.
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.
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.
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.
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.
When is cystic fibrosis screened?
At birth with the newborn blood spot test
What is often the first sign of CF?
Meconium ileus
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.
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.
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.
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.
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.
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.
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.
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.
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 isconsanguinity,
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.
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.
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.
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.
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.
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.
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.
Raised inflammatory markers (ESR and CRP) can indicate what abdominal conditions?
Watchman, Thomas. Zero to Finals Paediatrics (p. 21). Kindle Edition.
IBD
ANAEMIA can indicate what abdominal conditions?
IBD or coeliac
Raised anti-TTG or anti-EMA antibodies indicate what abdominal conditions?
Coeliac disease
Raised faecal calprotectin indicates what abdominal conditions?
IBD
Positive urine disptick indicates what abdominal conditions?
UTI
What is abdominal migrane?
Central abdominal pain lasting more than 1 hour
What is abdominal migrane associated with?
Nausea Anorexia Pallor Headache Photophobia Aura
Secondary causes of constipation?
Hirschsprung’s disease, cystic fibrosis or hypothyroidism.
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
Other rarer causes of encopresis include:
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
• Spina bifida • Hirschprung’s disease • Cerebral palsy • Learning disability • Psychosocial stress • Abuse
Watchman, Thomas. Zero to Finals Paediatrics (p. 23). Kindle Edition.
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.
Gastro-oesophageal reflux is
what?
Where the contents from the stomach reflux through thelower oesophageal sphincter into the oesophagus, throat and mouth.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 24-25). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 25). Kindle Edition.
Signs of problematic reflux include:
Watchman, Thomas. Zero to Finals Paediatrics (p. 25). Kindle Edition.
- Chronic cough • Hoarse cry • Distress, crying or unsettled after feeding • Reluctance to feed
- Pneumonia • Poor weight gain
Watchman, Thomas. Zero to Finals Paediatrics (p. 25). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 25). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 25). Kindle Edition.
In simple cases of GORD explanation, reassurance and practical advice is all that is needed. Advise:
Watchman, Thomas. Zero to Finals Paediatrics (p. 25). Kindle Edition.
• Small, frequent meals • Burping regularly to help milk settle • Not over-feeding • Keep the baby upright after feeding (i.e. not lying flat)
Watchman, Thomas. Zero to Finals Paediatrics (pp. 25-26). Kindle Edition.
More problematic cases of GORD can justify treatment with:
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
• Gaviscon mixed with feeds • Thickened milk or formula (specific anti-reflux formulas are available) • Ranitidine • Omeprazole where ranitidine is inadequate
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
What is torticollis?
forceful contraction of the neck muscles causing twisting of the neck
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
What is dystonia?
abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
What can happen after feeding in pyloric stenosis?
After feeding, there is increasingly powerfulperistalsisin the stomach as it tries to push food into the duodenum. Eventually it becomes so powerful that it ejects the food into theoesophagus, out of the mouth and across the room. This is called “projectile vomiting”.
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
How does pyloric stensois present?
typically presents in the first few weeks of life, with a hungry baby that is thin, pale and generallyfailing to thrive. The classicdescription of vomiting you should remember for your exams is “projectile vomiting”.
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
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 thehypertrophicmuscle of thepylorus.
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
Blood gas analysis in pyloric stensosi?
Blood gas analysis will show ahypochloric (low chloride) metabolic alkalosisas the baby is vomiting thehydrochloric acidfrom the stomach.
Watchman, Thomas. Zero to Finals Paediatrics (p. 26). Kindle Edition.
Management of pyloric stenosis?
Diagnosis is made using an abdominal ultrasound to visualise the thickened pylorus.
Treatment involves alaparoscopic 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.
What is acute gastritis?
is inflammation of the stomach and presents with nausea and vomiting.
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
What is enteritis?
inflammation of the intestines and presents with diarrhoea.
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
What is gastroenteritis?
inflammation all the way from the stomach to the intestines and presents with nausea, vomiting and diarrhoea.
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
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)
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
What does steatorrhoea suggest?
CF
Viral gastroenteritis is common. It is highly contagious. Common causes are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
Rotavirus
Norovirus
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 27). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 28). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 28). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 28). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 28). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 29). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 29). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 29). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 30). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 30). Kindle Edition.
What are autoantibodies created in response to in coeliac disease?
Gluten
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 30). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 30). Kindle Edition.
Symptoms of coeliac disease?
Failure to thrive Diarrhoea Fatigue Weight loss Mouth ulcers Anaemia secondary to iron deficiency Dermattis herpetiforms
Genes associated with coeliac disease
• HLA-DQ2 gene (90%) • HLA-DQ8 gene
Watchman, Thomas. Zero to Finals Paediatrics (pp. 30-31). Kindle Edition.
What should all patients with type 1 diabetes be checked for?
Coeliac disease
What autoantibodies are associated with coeliac diserase?
• Tissue transglutaminase antibodies (anti-TTG) • Endomysial antibodies (EMAs) • Deaminated gliadin peptides antibodies (anti-DGPs)
Watchman, Thomas. Zero to Finals Paediatrics (p. 31). Kindle Edition.
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”
Watchman, Thomas. Zero to Finals Paediatrics (p. 31). Kindle Edition. §
What is coeliac disease associated with?
• Type 1 diabetes • Thyroid disease • Autoimmune hepatitis • Primary biliary cirrhosis • Primary sclerosing cholangitis • Down’s syndrome
Watchman, Thomas. Zero to Finals Paediatrics (p. 31). Kindle Edition.
Complications of untreated coeliac disease?
• Vitamin deficiency • Anaemia • Osteoporosis • Ulcerative jejunitis • Enteropathy-associated T-cell lymphoma (EATL) of the intestine
Watchman, Thomas. Zero to Finals Paediatrics (p. 31). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 32). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 32). Kindle Edition.
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)
Watchman, Thomas. Zero to Finals Paediatrics (p. 32). Kindle Edition.
What is released by instestines when inflamed?
Faecal calprotectin
Gold standard investigation. for diagnosis of IBD?
Endoscopy
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 34). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 34). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 34). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 35). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 35). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 35). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 35). Kindle Edition.
What is Hrischprung’s disease?
a congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum.
Watchman, Thomas. Zero to Finals Paediatrics (p. 35). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 35). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 35-36). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 36). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 36). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 36). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 36). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 37). Kindle Edition.
What is intussusceptuon associated with?
• Concurrent viral illness • Henoch-Schonlein purpura • Cystic fibrosis • Intestinal polyps • Meckel diverticulum
Watchman, Thomas. Zero to Finals Paediatrics (p. 37). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 37). Kindle Edition.
What does recurrent jelly stools suggest?
Intussusception
Diagnosis of insussception?
US or constrast enema
Complications of intussusception?
• Obstruction • Gangrenous bowel • Perforation • Death
Watchman, Thomas. Zero to Finals Paediatrics (p. 37). Kindle Edition.
What is appenditis?
Inflammation of the appendix
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 38). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 38). Kindle Edition.
Complications of appendectomy
• Bleeding, infection, pain and scars • Damage to bowel, bladder or other organs • Removal of a normal appendix • Anaesthetic risks • Venous thromboembolism
Watchman, Thomas. Zero to Finals Paediatrics (p. 39). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 41). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 41). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 44-45). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 45). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 46). Kindle Edition.
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)
Watchman, Thomas. Zero to Finals Paediatrics (p. 46). Kindle Edition.
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)
Watchman, Thomas. Zero to Finals Paediatrics (p. 46). Kindle Edition.
The two pillars of correcting DKA are:
Watchman, Thomas. Zero to Finals Paediatrics (p. 46). Kindle Edition.
Correctdehydration evenlyover 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.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 46-47). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 47). Kindle Edition.
Adrenal insufficiaency features in babies?
• Lethargy • Vomiting • Poor feeding • Hypoglycaemia • Jaundice • Failure to thrive
Watchman, Thomas. Zero to Finals Paediatrics (p. 47). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 47-48). Kindle Edition.
The short synacthen test can be used to confirm
Watchman, Thomas. Zero to Finals Paediatrics (p. 48). Kindle Edition.
Adrebal insufficiency
Treatment of adrenal insufficiency is what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 48). Kindle Edition.
replacement steroids titrated to signs, symptoms and electrolytes.
Watchman, Thomas. Zero to Finals Paediatrics (p. 48). Kindle Edition.
What is addisonian crisis
an acute presentation of severe Addison’s, where the absence of steroid hormones result in a life threatening presentation.
Watchman, Thomas. Zero to Finals Paediatrics (p. 49). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 49). Kindle Edition.
What is congenital adrenal hyperplasia caused by?
is caused by a congenital deficiency of the21-hydroxylaseenzyme. This causesunderproduction ofcortisol and aldosteroneand overproduction ofandrogensfrom birth.
Watchman, Thomas. Zero to Finals Paediatrics (p. 49). Kindle Edition.
Genetic inhertiance of congenital adrenal hyperplasia?
Autosomal recessive
21-hydroxylaseis the enzyme responsible for what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 50). Kindle Edition.
convertingprogesteronetoaldosteroneandcortisol.
Converted to testosterone instead so high testosterone, low aldosterone and low cortisol
Watchman, Thomas. Zero to Finals Paediatrics (p. 50). Kindle Edition.
Female patients with CAH usually present how
Watchman, Thomas. Zero to Finals Paediatrics (p. 50). Kindle Edition.
presents at birth withvirilisedgenitalia, known as “ambiguous genitalia” and an enlarged clitoris due to the high testosterone levels.
Watchman, Thomas. Zero to Finals Paediatrics (p. 50). Kindle Edition.
Congenital growth hormone deficiency results from what
Watchman, Thomas. Zero to Finals Paediatrics (p. 51). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 51). Kindle Edition.
Growth hormone deficiency may present in neonates:
Watchman, Thomas. Zero to Finals Paediatrics (p. 51). Kindle Edition.
• Micropenis (in males) • Hypoglycaemia • Severe jaundice
Watchman, Thomas. Zero to Finals Paediatrics (p. 51). Kindle Edition.
Older infants and children can present with:
Watchman, Thomas. Zero to Finals Paediatrics (p. 51). Kindle Edition.
• Poor growth, severely slowing from age 2 - 3 • Short stature • Slow development of movement and strength • Delayed puberty
Watchman, Thomas. Zero to Finals Paediatrics (p. 51). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 52). Kindle Edition.
Congenital hypothyroidism is screened for on the newborn blood spot screening test. Where it is not picked up a birth, patients can present with:
Watchman, Thomas. Zero to Finals Paediatrics (p. 52). Kindle Edition.
• Prolonged neonatal jaundice • Poor feeding • Constipation • Increased sleeping • Reduced activity • Slow growth and development
Watchman, Thomas. Zero to Finals Paediatrics (p. 52). Kindle Edition.
The diagnosis of acute pyelonephritis is made if there is either:
Watchman, Thomas. Zero to Finals Paediatrics (p. 54). Kindle Edition.
• A temperature greater than 38°C • Loin pain or tenderness
Watchman, Thomas. Zero to Finals Paediatrics (p. 54). Kindle Edition.
What are leukocytes?
WBCs
What does presence of nitrites suggest?
Bacteria in urine
Management of vesico-ureteric reflux depends on the severity:
Watchman, Thomas. Zero to Finals Paediatrics (p. 55). Kindle Edition.
• Avoid constipation • Avoid an excessively full bladder • Prophylactic antibiotics • Surgical input from paediatric urology
Watchman, Thomas. Zero to Finals Paediatrics (p. 55). Kindle Edition.
Micturating cystourethrogram (MCUG) should be used to investigate what
Watchman, Thomas. Zero to Finals Paediatrics (p. 56). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 56). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 56). Kindle Edition.
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:
Watchman, Thomas. Zero to Finals Paediatrics (p. 56). Kindle Edition.
• 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
Watchman, Thomas. Zero to Finals Paediatrics (p. 56). Kindle Edition.
Nephrotic syndrome occurs when what?
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
Nephrotic syndrome features a classic triad of:
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
• Low serum albumin • High urine protein content (more than 3+ protein on urine dipstick) • Oedema
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
There are three other features that occur in patients with nephrotic syndrome:
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
• Deranged lipid profile, with high levels ofcholesterol, triglycerides and low density lipoproteins • High blood pressure • Hyper-coagulability, with an increased tendency to form blood clots
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
Most common cause of nephrotic disease in children?
Minimal change disease
Nephrotic disease can be secondary to intrinsic kidney disease such as
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
• Focal segmental glomerulosclerosis • Membranoproliferative glomerulonephritis
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
Management of minimal change disease?
corticosteroids (i.e. prednisolone). The prognosis is good and most children make a full recovery, however it may reoccur.
Watchman, Thomas. Zero to Finals Paediatrics (p. 57). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 58). Kindle Edition.
Complications of nephrotic syndrome?
- Hypovolaemiaoccurs as fluid leaks from the intravascular space into the interstitial space, causing oedema and low blood pressure
- Thrombosiscan 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
Watchman, Thomas. Zero to Finals Paediatrics (p. 58). Kindle Edition.
What is nephritis?
inflammation within the nephrons of the kidneys.
Watchman, Thomas. Zero to Finals Paediatrics (p. 58). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 58). Kindle Edition.
The two most common causes of nephritis in children
Watchman, Thomas. Zero to Finals Paediatrics (p. 58). Kindle Edition.
arepost-streptococcal glomerulonephritisandIgA nephropathy (Berger’s disease).
Watchman, Thomas. Zero to Finals Paediatrics (p. 58). Kindle Edition.
When does post strep glomerulonephritis occur?
1 - 3 weeks after a beta-haemolytic streptococcusinfection, such astonsillitis caused by Streptococcus pyogenes. Immune complexes made ofstreptococcal 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.
Watchman, Thomas. Zero to Finals Paediatrics (pp. 58-59). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 59). Kindle Edition.
What is IgA nephropathy also know as
Berger’s disease
What is the pathology of IgA nephropathy?
disease. This condition is related toHenoch-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”.
Watchman, Thomas. Zero to Finals Paediatrics (p. 59). Kindle Edition.
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.
Watchman, Thomas. Zero to Finals Paediatrics (p. 59). Kindle Edition.
When does Haemolytic uraemiac syndrome occur?
When there is thrombosis within small blood vessels throughout the body. Usually triggered by shiga toxin
Watchman, Thomas. Zero to Finals Paediatrics (p. 59). Kindle Edition.
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
Watchman, Thomas. Zero to Finals Paediatrics (p. 59). Kindle Edition.
Presentation of HUS
briefgastroenteritis, often with bloody diarrhoea. The symptoms of haemolytic uraemic syndrome typically start around 5 days after the onset of the diarrhoea.
Watchman, Thomas. Zero to Finals Paediatrics (p. 59). Kindle Edition.
