Paeds Flashcards
Normal obs in a neonate
Heart rate: 120-160
Resp rate: 30-60
BP: 60/30 - 90/60
Normal obs in infants, school age, adults
Infant
HR - 100-160
RR - 30-40
School age
HR - 70-120
RR - 18-30
Adult
HR 60-100
RR 12-20
What is epiglottitis and what is its main cause?
Life threatening emergency.
Swelling of epiglottis - can completely block airway in hours, typically caused by Haemophilus influenza B. (incidence decreased due to Hib vaccine)
Presentation of epiglottitis
Much more rapid onset than croup
- Fever, sore throat, stridor
- Drooling (painful throat prevents swallowing)
- Tripod position (sat forward with hands on knees - easier to breathe)
- Difficulty/painful swallowing and muffled voice
Investigations of epiglottitis
Direct visualisation by senior staff (to not distress patient)
Lateral X ray of neck shows “Thumb sign” (Swollen eipglottis pressing on trachea like a thumb)
Management of epiglottitis
- Immediate Senior Bleep to those able to provide airway support (Endotracheal Tube may be needed from anaesthetics, ENT etc).
- Oxygen
- IV Abx and steroids
Do not examine due to risk of airway obstruction
What is croup, and what is its most common cause?
Acute URTI causing oedema in the larynx of young children (6m-2y)
Parainfluenza virus most common
(AKA Laryngotracheobronchitis)
Presentation of croup
Usually preceded by non specific cough, rhinorrhoea etc.
- Harsh barking cough, worse at night
- Stridor (do not examine throat if stridor - may precipitate airway obstruction)
- Fever
- IWOB
Give signs of IWOB
- Nasal flaring
- Intercostal and subcostal recessions
- Tracheal tug
- Use of accessory muscles
- Head bobbing
- Grunting
- Increased resp rate
Investigations of croup
- Clinical diagnosis (dont examine throat)
- Posterior-anterior X Ray - steeple sign (subglottic narrowing)
Management of Croup
Single oral dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity.
Should resolve in 48 hours.
If not, or if severe, oxygen + Nebulised adrenaline
What cause of Croup goes on to cause epiglottitis
Croup caused by diphtheria causes epiglottitis and has a high mortality
Causes of HAP
Early (<5 days admission) - S pneumoniae
Late (>5 days admission) - S aureus, gram negative bacteria (P aeruginosa, H influenzae)
Causes of CAP (bacterial, viral and fungal)
Bacterial
- S pneumoniae (most common)
- H influenzae
- S aureus
Viral
- Influenza virus
- Parainfluenza
- RSV
Fungal
- Chlamydia trachomatis
Management of pneumonia in children
- Amoxicillin first line.
- Macrolides used if jirovecii or chlamydia.
- Co-amoxiclav if influenza
Most commonly affected lobe in pneumonia
Right middle/lower due to it being wider and more vertical than left bronchus, facilitating aspirate passage
What is pneumocystis pneumonia?
Unicellular eukaryote - opportunistic infection in AIDS. Causes extra pulmonary manifestations (Hepatosplenomegaly, lymphadenopathy and choroiditis)
What is bronchiolitis? What is its main cause?
Acute bronchiolar inflammation usually caused by RSV. Most common LRTI in under 1s.
Maternal IgG from breast feeding usually provides protection against RSV
Risk factors for bronchiolitis
- Formula fed, or breastfed <2 months (less maternal IgG)
- Smoke exposure
- Immunodeficiency
- Siblings in school
How does bronchiolitis present
- Dry cough
- Increasing breathlessness
- Wheezing w/ fine inspiratory crackles
- Difficulties feeding due to dyspnoea
- Coryzal symptoms (fever, rhinorrhoea, blocked nose, watery eyes)
When should referral be considered in bronchiolitis? And when is it immediately urgent
- Resp rate >60
- Inadequate intake or clinical dehydration
Urgent
- Apnoea
- Severe resp distress
- Central cyanosis
- O2 sats <92
- Unwell looking
How is bronchiolitis investigated and treated?
Clinical diagnosis, nasopharyngeal secretions may show RSV
Supportive management
- Humidified oxygen via nasal cannula if O2 <92%
- NG tube feeding
- Suction if excessive upper airway secretions
What is Cystic Fibrosis
A phenylalanine deletion on the CFTR (CF transmembrane conductance regulator) gene on chromosome 7, causing secretions to become much thicker. Mainly affects pancreas and lungs.
Autosomal recessive.
How does CF affect the pancreas and the lungs
Pancreas - thick pancreatic and biliary secretions block ducts, causing pancreatitis (autodigestion), cholangitis and lack of digestive enzymes in GI tract.
Causes failure to thrive, steatorrhoea and endocrine dysfunction e.g. CF diabetes.
Lungs - Impaired mucociliary clearance. Causes an obstructive pattern and thick immobile secretions = repeat S aureus and P aeruginosa infections.
Earliest presentation of CF in neonates?
Meconium ileus - Meconium isnt passed and instead causes a blockage in the intestine.
Clinical features of CF
- Meconium ileus
- Chronic cough, wheeze and recurrent infections (S aureus, P aeruginosa and H influenza)
- Nasal polyps and sinusitis
- Pancreatic insufficiency (steatorrhoea, malabsorption)
- GORD
- Clubbing
Investigations of CF
- Newborn guthrie heel prick screening (Immunoreactive trypsinogen)
- GOLD: Chlorine sweat test. Pilocarpine induces sweating. Sweat has more chlorine (>60mmol/L)
- Faecal elastase (pancreatic insufficiency)
CF Lifestyle advice
- High calorie, high fat diet
- No smoking
- Regular exercise and physio
- Flu vaccines
Respiratory management of CF
- Chest physio and exercise
- Bronchodilator (Salbutamol)
- Mucolytic (Dornose Alfa)
- Nebulised Tobramycin if pseudomonas
- Prophylactic flucloxacillin for life
Digestive management of CF and problems caused by it (5)
- High calorie high fat diet
- Fat soluble vitamins (ADEK)
- Pancreatic enzyme replacement (Creon)
- PPI - omeprazole
- Ursodeoxycholic acid (make bile more soluble)
Complications of CF + life expectancy
Life expectancy <40
CF related diabetes
Liver/biliary cirrhosis
Recurrent URTI
Malabsorption
- Delayed puberty
- Osteoporosis
- Infertility
What is otitis media and what is it caused by?
Inflammation of the middle ear (between tympanic membrane and oval window).
Most commonly secondary to viral URTI (RSV and rhinovirus) but ear infection is bacterial (S. pneumoniae, Moraxella catharallis, H influenzae, S aureus)
How does Otitis media present and what would you see on examination?
- Earache and tugging on ear
- Fever, hearing loss, discharge
On examination
- Tympanic membrane is red and bulging outwards. Loss of light reflex
- May be perforation and discharge
Management of Otitis media
Usually self limiting (3 days)
Give Abx if:
- >4 days and no improvement
- Systemically unwell
- immunocompromised
- If under 2 and bilateral otitis media
- Discharge or perforation
Define otitis externa with some main risk factors and causative bacteria
- Infection of the outer ear canal.
- Swimming, hot/humid climate, trauma, use of hearing aids/earplugs
- P aeruginosa, S aureus.
Presentation and examination of otitis externa
- Ear pain, itching, hearing loss, fullness in ear
- Tender pinnus/tragus, normal tympanic membrane
What is glue ear? What can unilateral glue ear suggest?
Otitis Media with Effusion. Chronic ear infection or eustachian tube dysfunction can cause a build up on viscous inflammatory fluid, causing conductive hearing loss.
Unilateral OME can suggest a middle ear tumour.
How does glue ear normally present and what do you see on examination?
- Conductive hearing loss and aural fullness
- Dull grey tympanic membrane, lack of light reflex, and a bubble trapped behind tympanic membrane
Management of glue ear
Most cases resolve in 3 months. Otherwise, non surgical (hearing aid, autoinflation) or surgical intervention may be needed (Myringotomy and grommet insertion)
What is a grommet, and how is it used in glue ear
Negative pressure in middle ear causes fluid accumulation leading to glue ear. A grommet is a small pipe that is placed into the tympanic membrane to keep pressure the same on either side by allowing air to enter the middle ear.
It is inserted after a myringotomy (incision in tympanic membrane to drain fluid in middle ear).
How long do grommets stay in? What is some lifestyle advice to avoid infection
6-12 months, but some can be long term.
Avoid water! and regular ENT check ups
Give the two types of deafness with possible causes
Conductive - When sound waves not conducted through outer ear to eardrum. Causes: eardrum perforation, fluid in middle ear, earwax buildup.
Sensorineural - Damage to inner ear (cochlea) or to nerve pathways from ear to brain. Causes: Aging, loud sound exposure, Meniere’s disease.
How can the deafnesses be differentiated on examination
Conductive
- Rinnes: Bone conduction > Air conduction (negative result)
- Webers: Heard more in the bad ear
Sensorineural
- Rinnes: Air conduction > Bone conduction (Positive - Normal result)
- Webers: Heard more in the good ear
What are the most common causes of deafness
Ear wax, otitis media, otitis externa
What is otosclerosis, what are 3 features
Autosomal dominant replacement of normal bones in ear with spongy vascular bone.
- Conductive deafness
- Tinnitus
- “Flamingo tinge” to tympanic membrane
- Family history
What causes a post viral wheeze?
Bronchoconstriction in response to inflammation and oedema following an RSV or Rhinovirus infection
Who does post viral wheeze normally affect, and how is it managed
Preschoolers <3 years
SABA, nebulised ipratroprium bromide or 5 days oral prednisolone
Define orbital cellulitis with its main cause
Infection of fat and muscle cells posterior to orbital septum. Commonly due to URTI from sinuses and is a medical emergency with high mortality.
Caused by S aureus, HiB and streptococcus.
Risk factors for orbital cellulitis
- Childhood (7-12)
- Previous sinus infection
- Lack of Hib immunisation
- Recent eyelid infection
- Ear/face infection
Presentation of orbital cellulitis
- Swelling/redness of eye
- Severe ocular pain, worse on movement
- Reduced acuity
- Eyelid oedema and ptosis
- Exophthalmos (AKA Proptosis)
Differentiation between orbital and periorbital cellulitis
Periorbital/preseptal does not have reduced visual acuity or pain with eye movement.
Imaging used for orbital cellulitis
CT with contrast - look for inflammation of orbital tissue
Management of orbital cellulitis
Hospital admission and 7-10 days of IV antibiotics - Cefotaxime
Define preseptal/periorbital cellulitis and who is it more common in
Preseptal is when the infection is anterior to orbital septum. Includes eyelids, skin and subcutaneous tissue but does not affect the orbit or its contents.
More common in kids under 21 months
Causes of preseptal cellulitis (causative bacteria)
- S aureus
- S epidermidis
- Streptococcus
presentation and management of periorbital cellulitis
- Red swollen surrounding tissue of eye
- NO visual disturbances or affect to eye muscle.
Managed with oral co amox
What is squint and how is it investigated
Eyes pointing different directions
AKA Strabismus
- Misalignment of the visual axes. Can be concominant (imbalance in extraocular muscles - Convergent more common than divergent) or because one is paralysed (rare, due to paralysis of extraocular muscles)
Detected using corneal light reflex to check symmetry of light reflection
How does an innocent (flow) murmur sound?
- Soft
- Short
- Systolic
- Symptomless (no thrill, added sounds, cyanosis, SOB etc)
- Situational (quieter when standing, only when unwell etc)
Features of a murmur that suggest bad murmur
- Louder than 2/6
- Diastolic
- Louder standing
- Symptoms (failure to thrive, feeding difficulty, cyanosis, SOB)
Where are the pan systolic murmurs heard
Mitral regurgitation - Mitral (5th intercostal, mid clavicular)
Tricuspid regurgitation - Tricuspid (5th intercostal, left sternal edge)
Ventricular septal defect - Left lower sternal border
Where are the ejection systolic murmurs heard
Aortic stenosis - Aortic (2nd intercostal, right sternal edge)
Pulmonary stenosis - Pulmonary (2nd intercostal left sternal edge)
Hypertrophic obstructive cardiomyopathy - (4th intercostal left sternal edge - just above tricuspid)
What is patent ductus arteriosus, give risk factors
Abnormal connection between descending aorta and pulmonary artery. Usually close with first breaths. Aorta has greater pressure than pulmonary vessels, causes a left to right shunt, causing pulmonary hypertension and right heart strain and RVH. Increased pressure then causes LVH
- Prematurity
- Born at high altitude
- Maternal rubella infection
Murmur and features on examination associated with patent ductus arteriosus
Continuous crescendo-decrescendo “machinery” murmur.
- Left subclavicular thrill
- Large volume, bounding, collapsing pulse
- Wide pulse pressure
- Apex heave
Management of PDA
Indomethacin or ibuprofen - usually closes the connection in neonates. Given after 1 week, to allow time to close on its own
Monitoring until 1yo
What sound does an atrial septal defect make
Mid (AKA ejection) systolic, crescendo-decrescendo murmur loudest at upper left sternal edge. Also has a fixed split heart sound (doesn’t change with breathing)
Pathophysiology of atrial septal defect
Blood shunts from left atrium to right. Hence, blood flows to lungs (ACYANOTIC) but increased right heart flow causes right heart overload and strain. Can lead to right heart failure and pulmonary HTN.
Most common congenital heart defect in adults, 50% dead by 50
How can pulmonary HTN cause Eisenmenger syndrome
Pulmonary HTN increases so much the pulmonary pressure > systemic pressure. Reverses the L2R shunt and causes it to become R2L, so blood bypasses lungs and becomes cyanotic.
Give the 2 main types of ASD
Ostium Secondum (70%, associated with Holt-Oram syndrome (tri-phalangeal thumbs))
Ostium primum (associated with abnormal AV valves)
What are ventricular septal defects
Most common congenital heart disease. 50% close spontaneously.
- Congenital VSDs are associated with chromosomal disorders (Down’s, Edwards, Pataus etc) and congenital infections.
How are most VSDs detected
In utero (20 wk scan). Post natally, failure to thrive, features of heart failure, pan systolic murmur.
What is transposition of the great arteries
Most common CHD at birth. Cyanotic heart disease in which aorta leaves the right ventricle and pulmonary artery leaves left ventricle. (positions switched).
Causes oxygenated blood to flow round the lungs, and deoxygenated blood to flow around the body
Investigation signs of ToGA
- Loud single S2
- Prominent RV impulse
- “egg-on-side” appearance on CXR
Management of ToGA
- Maintaining the ductus arteriosus with prostaglandins to flow oxygenated blood from the aorta to pulmonary artery for oxygenation
- Surgical is definitive
4 congenital abnormalities in Tetralogy of Fallot
VORP - cause right to left cardiac shunt; blood bypasses lungs.
Ventricular Septal Defect - Blood shunts between ventricles. Oxygenated and deox. mix. Deox more into left than ox into right.
Overriding Aorta - Aorta further right than normal. RV sends deox blood into it
RV Hypertrophy - Due to added resistance of LV, ensures deox blood is shunted to left, rather than the other way.
Pulmonary stenosis - RV outflow obstruction makes it harder for deox blood to reach lungs
What is tetralogy of fallot
4 congenital abnormalities causing an increase in deoxygenated blood around the body. Causes cyanosis, and the child may develop hypercyanotic “tet” spells with increased need (crying, feeding etc), causing a “squatting position”, resp distress, syncope, tachypnoea.
Risk factors for TOF
- Family history
- Diabetic mum
- Down’s
- Alcohol in pregnancy
- Rubella
X ray and ECHO signs of TOF
X - Boot shaped heart
Echo - RV hypertrophy
Treatment of TOF and tet spells
Surgical repair
Tet spells - Beta blockers, oxygen, morphine, sodium bicarbonate, phenylephrine
What is pulmonary atresia
Pulmonary valve doesn’t form properly, meaning blood cant reach lungs. Requires alternative pathway to lungs.
What is Perthes Disease
Degenerative hip joint condition affecting 4-8yo. Due to avascular necrosis of the femoral head (femoral epiphyses), followed by revascularisation and reossification over 18-36 months. 5x more common in boys
Signs and timeframe and XRay of Perthes Disease
- Progressive hip pain over weeks without trauma
- Limp, stiff, reduced hip movement
Early- Widened joint space
Later- Decreased femoral head size/flattening, sclerosis
Management and complications of Perthes
Most resolve with conservative management, e.g. bracing to keep femoral head in place. If >6 yo, or severe deformity, surgery.
- Osteoarthritis
- Premature fusion of growth plates
What is rheumatic fever and what can repeat exposure cause. What type of hypersensitivity is it
Type 2 hypersensitivity to a recent strep pyogenes infection (strep throat - 2-4 weeks after).
Rare in west, causing joint pain and carditis. Repeat exposure can cause fibrosis of valves, causing regurgitations
What can be found histologically on the hears of people with rheumatic fever
Aschoff bodies (granulomatous bodies)
Diagnostic criteria for rheumatic fever
Revised Jones criteria (JONES-FEAR)
Evidence of recent infection (group A antigen test, positive throat culture, strep antibodies (ASO antibody titre)) + 2 major signs or 1 major 2 minor.
- Joint arthritis
- Organ inflammation (carditis+murmur)
- Nodules under skin (firm, painless)
- Erythema marginatum (red splodgy rash all over)
- Sydenham’s chorea
- Fever
- ECG (prolonged PR)
- Athralgia without arthritis
- Raised ESR/CRP
What valve diseases does rheumatic fever cause
Acute - Mitral and aortic regurgitations
Chronic - Mitral stenosis
Management of rheumatic fever
Oral penicillin V for 10 days and NSAIDs
Define GORD in infants
Commonest cause of vomiting in infancy. Normal to regurgitate feeds but GORD when it starts to cause distress. Caused by a weak sphincter which normally resolves by 1 year
Presentation of GORD in children
Signs of distress more than usual post feed vomiting
- Chronic cough
- Hoarse, excessive crying, especially while feeding
- Milky vomits and regurgitation after feeds or after being laid flat
- Poor weight gain
Gastro red flags in children and what they may mean
- Not keeping feeds down +- projectile/forceful vomiting (pyloric stenosis or intestinal obstruction)
- Bile stained vomit and/or abdominal distention (intestine obstruction)
- Haematemesis or malaena (peptic ulcer, oesophagitis, varices)
- Respiratory symptoms (aspiration/infection)
- Rash, angioedema (milk protein allergy)
- Apnoeas
Management of GORD in children
Usually doesn’t need medical therapy.
- 30 degree head up position during feeds
- Sleep on back
- Ensure not overfeeding, and burp regularly
- Thickened formula
- Gaviscon with feeds if bad
What is Sandifer’s syndrome
A rare condition causing brief episodes of abnormal movements usually associated with GORD
- Torticollis: Forceful contraction of the neck muscles causing twisting of the neck
- Dystonia: Abnormal muscle contractions causing twisting movements, arching of the back.
Usually self resolves but important differential is infantile spasms and seizure
How does pyloric stenosis present and what is it caused by? What are associated electrolyte changes
Usually presents in 2nd to 4th weeks of life, with
- Projectile vomiting (~30mins after a feed)
- Constipation and hydration
- Palpable mass in upper abdo
- Hypochloraemic, hypokalaemia alkalosis due to persistent vomiting.
Caused by hypertrophy of the circular muscles of the pylorus
How is pyloric stenosis diagnosed and managed
Diagnosed with ultrasound
Managed with Ramstedt pyloromyotomy
What is Coeliac disease
A sensitivity to the gluten protein. Repeated exposure leads to villous atrophy causing malabsorption. Children normally present before 3yo. Normally affects small bowel, particularly the jejunum
How does coeliac present
Often asymptomatic, low threshold for testing
- Failure to thrive
- Diarrhoea
- Abdominal distention
- Anaemia
- Mouth ulcers
- Dermatitis herpetiformis - itchy, blistering skin rash that typically appears on the abdomen
Genetic association with coeliac disease
HLA-DQ2 (90%)
HLA-DQ8
Investigations for Coeliac
Antibodies
- Anti-TTG and Endomysial antibodies (IgA - undetectable if IgA deficiency)
- Anti-DGPs (Deaminated Gliadin Peptides)
Endoscopy and intestinal
- Crypt hyperplasia
- Villous atrophy
- Increased intraepithelial lymphocytes
- Lamina propia infiltration
Gluten free diet trial
Treatment of Coeliac
Gluten free diet
Possible complications of untreated coeliac
- Malnutrition/vitamin deficiency
- Anaemia
- Osteoporosis
- Non hodgkins lymphoma
- Small bowel adenocarcinoma
Disease associations with coeliac
Autoimmune
- T1DM
- Thyroid disease
- Autoimmune hepatitis
- PBC/PSC
- Down’s
Causes of childhood constipation
Idiopathic or functional - no underlying cause
Secondary:
- Hypothyroidism
- Hirschsprung’s Disease
- Cystic fibrosis
- Hypercalcaemia
- Dehydration
- Low fibre diet
- Medication induced e.g. opiates
How does constipation present in children and what posture might they take?
- Less than 3 stools a week
- Hard stool difficult to pass
- Rabbit dropping (bristol stool type 1)
- Abdominal pain, waxes and wanes with passage of stool. Possibly a palpable abdominal mass
- Overflow soiling
- Poor appetite
- Straining, pain, bleeding
Retentive posturing
- Straight legged
- Tiptoed
- Back arched
Red flag symptoms suggesting underlying disorder in childhood constipation
- Reported from birth or first weeks of life
- Meconium took >48 hours
- Ribbon stool pattern
- Weakness in legs
- Faltering growth
- Distension
- Disclosure or evidence of mistreatment
At what age is faecal incontinence considered pathological?
4 years old.
Usually a sign of chronic constipation, causing stretching and desensitisation of the rectum.
Indications of idiopathic constipation
- Start after first few weeks of life, obvious precipitating factors
- Meconium in less than 48h
- Growth normal
- No neurological deficit or problems
- Changes in diet
Management of constipation in kids, including if there is impactions, or if the child is <6 months
Diagnosis of idiopathic can be made if no red flags
- Movicol Paediatric Plan first line (contains Polyethylene glycol 3350 and electrolytes)
- Add stimulant laxative if no disimpaction (Senna)
- Lifestyle advice: High fibre, hydration, exercise, reduce risk factors
If Impaction:
- May need disimpaction regime with high dose of laxatives
<6 months
- Bottle fed: Water between feeds, gentle massaging and bicycling legs
- Breast fed: Unusual to be constipated when breast fed
Non pharmacological management of constipation
Encourage toilet visits
- Scheduling visits
- Bowel diary
- Star charts
What are possible causes of diarrhoea and vomiting in children
- Infection (gastroenteritis)
- IBD/IBS
- Lactose intolerance
- Coeliac
- Cystic fibrosis
Viral causes of gastroenteritis
Rotavirus
Norovirus
Highly common and contagious
Adenovirus possible but more subacute
What does E coli cause
E coli is a normal intestinal bacteria. Spread through contact with contaminated faeces, unwashed salad and contaminated water.
E coli 0157 produces shiga toxin, causing cramps, bloody diarrohea, vomiting. Shiga toxin destroys bloody cells and leads to haemolytic uraemic syndrome.
Abx increase risk of HUS so avoid if E coli.
What does C jejuni cause and how does it look histologically
Travellers diarrhoea. Most common cause of bacterial gastroenteritis worldwide. Gram negative, curved/spiral
Spread through
- Raw/undercooked chicken
- Untreated water
- Unpasteurised milk
Incubation 2-5 days and 3-6 days of symptoms
- Abdominal cramps
- Bloody diarrhoea
- Vomiting and fever
Azithromycin or ciprofloxacin if severe
What does shigella cause, and how is it spread, and how is it treated
Spread through faeces contaminated drinking water, swimming pools, food. Incubation 1-2 days, symptoms usually resolve within a week without treatment.
Bloody diarrhoea, cramps, fever.
Shigella produces the shiga toxin that causes HUS.
Azithromycin or ciprofloxacin in severe cases
What does salmonella cause
Spread through raw eggs or chicken, or food contaminated with small animal poo. Symptoms usually resolve in a week.
Watery diarrhoea that can be associated with blood or mucus, abdo pain, vomiting. Abx only in severe cases
What are some other infective causes of diarrhoea
(fried rice, pork, parasitic infection)
Bacillus Cereus - Fried rice left at room temp. Sx start in 5 hours, resolves in 24 hours. Vomiting first and diarrhoea.
Yersinia Entercolitica - Gram negative bacillus, spread through pork. Affects children. Key symptom is lymphadenopathy. In adults this causes right sided mesenteric lymphadenopathy (mimicking apendicitis)
Giardiasis - Microscopic parasite. Live in small intestine of mammals, releasing cysts in their stools, which contaminate food and water. Treated with metronidazole
Staph aureus toxin
Produces enterotoxins on food such as eggs, dairy and meat. Cause intestine inflammation, causing diarrhoea, profuse vomiting, abdo cramps and fever. Sx settle in 12-24 hours
What does chronic diarrhoea in an infant suggest?
Cows’ milk intolerance
How does clinical shock due to dehydration present
- Decreased consciousness
- Cold extremities
- Pale skin
- Tachycardia and tachypnoea
- Weak peripheral pulses
- Prolonged cap refill
- Hypotension
Symptoms of hyponatraemic dehydration
Jittery movements
Increased muscle tone
Hyperreflexia
Convulsions
Drowsiness/coma
How to rehydrate dehydration
- 50ml/kg low osmolarity oral rehydration solution over 4 hours.
What is irritable bowel syndrome, how does it present and how long should symptoms last
Abdominal pain or
Bloating or
Change in bowel habit and stool constitution (watery, loose, hard, mucus)
Pain relieved with defecation.
> 6 months.
Diagnosed through exclusion and if
- Altered stool passage
- Abdominal bloating
- Symptoms made worse by eating
- Passage of mucus
Red flags in bloody diarrhoea
- Rectal bleeding
- Unexplained weight loss
- FH of bowel or ovarian cancer
- > 60y onset
Investigations in IBS, including cancers that mimic it (not necessarily in kids)
- FBC (check anaemia)
- Inflammatory markers
- Coeliac serology
- Faecal calprotectin
- CA125 (ovarian cancer) CA19/9 (pancreatic)
*Ovarian presents similar, just much older. E.g. 50yo woman with bloating, abdo pain. Non specific
Non medical management of IBS
Lifestyle advice
- Fluids
- Regular small meals
- Low FODMAP diet
- Limit caffeine, alcohol, fatty foods
- Reduce stress
- Change fibre depending if constipation or diarrhoea
CBT may be used
Medical management of IBS
- Loperamide for diarrhoea (slows down bowel)
- Linaclotide for constipation (laxative)
Pathophys of appendicitis
Appendix sits at end of caecum. Trapped stool or other obstruction causes infection and inflammation, which quickly causes gangrene and rupture. Rupture releases contents into abdomen, causing peritonitis and possibly sepsis.
How does appendicitis present
- Severe central abdominal pain that moves to RIF over time.
- Tenderness in McBurney’s point (2/3 of the distance from belly button to ASIS (Anterior Superior Iliac Spine))
- Rovsing’s sign (palpation of left iliac fossa causes pain in RIF)
- Guarding
Rebound tenderness and percussion tenderness if progression to peritonitis
How is appendicitis diagnosed
Usually made clinically:
- Pain moving centrally to RIF
- low grade pyrexia
- minimal vomiting
CT GOLD Standard
Ultrasound in women (rule out ovarian cancer and gynae pathology)
Diagnostic laparoscopy if negative, with appendectomy in same procedure
Key appendicitis differentials
Ectopic pregnancy - bHCG will be raised (test to rule out)
Ovarian cysts - Pelvic and IF pain, not always right, can rupture or torsion
Meckels diverticulum - Malformation of distal ileum, usually asymptomatic but can inflame, rupture, bleed, volvulus, intussusception.
Mesenteric adenitis - Inflamed lymph nodes. Often associated with tonsilitis or URTI
Appendix mass - Omentum surrounds and sticks to inflamed appendix. Treated with abx and supportively. Appendectomy once acute infection resolves
Complications of appendectomy
- Bleeding, infection, pain scars
- Bowel adhesions, causing obstruction
- Damage to nearby organs
- Veneous thromboembolism
What is bowel intussusception, what age does it normally affect
When a portion of the bowel invaginates into the lumen on the adjacent bowel, most commonly around the ileo-caecal region. This increases bowel size and narrows lumen, causing ostruction.
Usually affects infants 6-24 months. Boys 2x as often
Presentation of intussuception. What does the infant do during attacks?
- Intermittent severe crampy progressive abdo pain
- Inconsolable crying
- During attack the infant will raise knees up and turn pale
- Vomiting
- Bloodstained stools “red-currant jelly” late sign
- Sausage shaped mass in upper right quadrant
May present with viral URTI preceding illness
Investigation and management of bowel intussusception
Ultrasound is investigation of choice - target like mass
- Managed with reduction by air insufflation.
- OR Barium enema
Complications of intussusception
Obstruction
Gangrene
Perforation
Death
What is Meckel’s diverticulum and how does it present. What is a complication it’s known for? What happens to its mucosa?
Congenital diverticulum of the small intestine. Remnant of omphalomesenteric duct and contains ectopic ileal, gastric or pancreatic mucosa
Usually asymptomatic but can present with abdominal (RLQ) pain, rectal bleeding or intestinal obstruction. It is the biggest cause of painless massive GI bleeding between 1 and 2y.
How is Meckels diverticulum investigated and managed
Meckels scan
- 99m Technetium pertechnetate scan, which has an affinity for gastric mucosa
Managed
- Laparoscopic surgery or diverticulectomy.
- Small bowel resection and anastomosis.
Rule of 2s
Meckel’s diverticulum
- 2% of population
- 2 inches long
- 2 feet from ileocaecal valve
- 2x as likely in boys
- 2yo when it causes GI bleed
- 2 types of ectopic tissue (pancreatic and gastric (susceptible to ulcer))
What is hirschsprung’s disease
Congenital absence of nerve cells of the parasympathetic myenteric plexus are absent in the distal bowel and rectum.
Known as Auerbach’s and Meissner plexuses.
Causes uncontrolled peristalsis, effectively being an obstruction.
Associations of Hirschprung’s
3x more common in males
Downs syndrome
Neurofibromatosis
Waardenburg syndrome
Presentation of Hirschprung’s
Delay in passing meconium
Chronic constipation
Abdo pain/distension
Vomiting
Poor weight gain and failure to thrive
How is Hirschprung’s investigated and managed
Abdominal X ray
Rectal biopsy GOLD
Managed
- Rectal washout/bowel irrigation
- Definitive: Surgery to affected colon segment
What is a Hirschprung associated complication
Hirschsprung-Associated Enterocolitis
Inflammation and obstruction of the bowel. 2-4 weeks of birth with fever, abdo distension, bloody diarrhoea, features of sepsis.
Can lead to toxic megacolon and perforation
IV Antibiotics, fluid resus, decompression of bowel
What is biliary atresia
Blockage or absence of extrahepatic bile duct, causing jaundice due to high conjugated bilirubin, as it is not being excreted in the bowel.
What are the types of biliary atresia
Type 1: Proximal ducts present, but common duct obliterated
Type 2: Atresia of the cystic duct, and systic structures found in porta hepatis
Type 3: Atresia of left and right ducts to the level of the porta hepatis (most common)
How does biliary atresia present
- Jaundice extending beyond physiological 2 weeks
- Dark urine and pale stools
- Appetite and growth disturbance
- Hepatosplenomegaly
Investigations for biliary atresia
- Unconjugated and conjugated bilirubin: Bilirubin may or may not be raised, but conjugated will be raised for sure.
- Ultrasound of biliary tree
- Percutaneous liver biopsy/intraoperative cholangioscopy
Management and complications of biliary atresia
Surgical - Kasai portoenterostomy (attach small intestine to liver opening, allowing bile excretion)
- Progressive liver disease
- Cirrhosis
- HCC
If surgery unsuccessful, may need transplant
What can cause jaundice in the first 24 hours? How about between 2-14 days
First 24 always pathological
- Rhesus haemolytic disease
- ABO haemolytic disease
- Hereditary spherocytosis
- G6PD Deficiency
2-14 days
- Usually physiological and associated with breastfeeding
Causes of prolonged neonatal jaundice (>14 days)
- Biliary atresia
- Hypothyroid
- Galactosemia
- UTI
- Breast milk jaundice
- Prematurity
What is cows milk protein allergy/intolerance
Usually seen in the first 3 months of formula fed children,
can rarely be seen in exclusively breastfed infants.
CMPA for immediate (<2 hours, IgE mediated)
CMPI if mild/moderate and delayed (several days, non IgE mediated)
Features of CMPI/A
Occurs in first 3 months of life:
- Regurgitation and vomiting
- Diarrhoea
- Urticaria
- Colic symptoms (crying, irritability)
- Wheeze, chronic cough
- Rarely anaphylaxis
Cows milk protein intolerance has the bloating, wind, diarrhoea and vomiting, but no rash, angio-oedema, sneezing or coughing.
How is CMPA investigated
skin prick/patch testing
total IgE and specific IgE (RAST) for Cows milk protein
Avoiding cows milk should fully resolve issues
Management of CMPA
Avoid cow’s milk
If formula fed
- Extensive hydrolysed formula first line replacement
- If intolerance, can be started on milk ladder
Breast feeding
- Breast feeding mother should avoid dairy products
- May need maternal calcium supplements
- Hydrolysed formula milk when breastfeeding stops until 12 months, for at least 6 months
Possible causes of intestinal obstruction
- Meconium ileus
- Hirschprung’s disease
- Oesophageal/duodenal atresia
- Intussusception
- Intestinal malrotation
Presentation of intestinal obstruction
- Persistent vomiting
- Abdominal pain/distention
- Failure to pass stool/wind
- Bowel sounds (tinkling early and absent later)
Investigations in bowel obstruction
Abdominal X ray
- Dilated loops of bowel proximal to obstruction and collapsed distal.
- Absence of air in rectum
Management of bowel obstruction
- NBM
- NG Tube and draingage
- IV fluids
- Surgical definitive
What is Toddler’s diarrhoea
Chronic, non specific diarrhoea, between 1 and 4 years of age. Is a diagnosis of exclusion
Features of toddlers diarrhoea
Frequent, poorly formed, offensive stools
Food material easily recognisable
Child is normally well, active and has unimpaired growth. Normal appetite and normal/increased fluid intake.
No positive findings on examination or lab investigations
Management of toddlers diarrhoea
Paternal reassurance, avoidance of full strength fruit juice.
Faeces normally becomes firm when child is toilet trained or by 3 years
What is failure to thrive
Poor physical growth and development
Faltering growth if they fall in centile spaces (e.g. distance between 75th and 50th centile)
- 1 if birthweight <9th centile
- 2 if birthweight 9-91st centile
- 3 if birthweight above 91st centile
Causes of failure to thrive
Inadequate intake
- Maternal absorption
- Neglect
- Poverty
Difficulty feeding
- Poor suck (cerebral palsy)
- Cleft lip/palate
- Pyloric stenosis
Malabsorption
Increased energy requirements
- Hyperthyroid
- Cystic fibrosis, heart disease
- Chronic infection
Inability to process nutrition
How do BMI and Mid parental height suggest inadequate growth
- Height more than 2 centiles below mid parental height centile
- BMI below 2nd centile
What is Crohn’s disease
Chronic inflammation of the entire GI tract (mouth to anus). Terminal ileum and colon most commonly affected. Affects white people more
Risk factors for Crohns
Family history
Smoking
White people
OCP
Diet low in fibre
NSAID
Genetic association with Crohns
NOD2/CARD15 gene mutation
What is the inflammation pattern of crohns
Transmural inflammation with areas of healthy bowel in between known as skip lesions, giving a cobblestone appearance.
Signs and symptoms of Crohns (Think LOWER GI Tract)
- Aphthous mouth ulcers
- Abdominal tenderness and diarrhoea
- Perianal lesions- skin tages, fissures, abscesses, ulcers, etc
- Weight loss and failure to thrive
Extra intestinal manifestations of Crohns
Skin
- Perianal/mouth ulcers
- Erythema nodosum
- Pyoderma gangrenosum
MSK
- Arthritis
- Seronegative spondyloarthropathy
- Clubbing
Eyes:
- Conjunctivitis
- Iritis
Investigations in Crohn’s
Faecal calprotectin raised
Serology
- pANCA Negative (more in UC)
- ASCA positive (more in Crohns)
Endoscopy + Biopsy
- Endoscopy: Skip lesions, cobblestoning, strictures
- Biopsy: Transmural inflammation, non caseating granulomas, goblet cells present
Small bowel enema
- High sensitivity/specificity for examination of terminal ileum
- Stricturs (Kantor’s string sign)
- Proximal bowel dilation
- Rose thorn ulcers
- Fistulae
Treatment of Crohns
Stop smoking, NSAID
Induce remission
- Glucocorticoids: Budesonide (mild), prednisolone (moderate), IV hydrocortisone (very severe)
- Immunosuppressant: Azathioprine or methotrexate
- AntiTNF (infliximab)
Maintain remission:
- Azathioprine
Indications for surgery in crohns
80% of patients
- Sticturing terminal ileus disease (ileocaecal resection)
- Perianal fistulae (Diagnosed with MRI, treated surgically and draining seton left in to prevent abscess formation)
- Perianal abscess (Surgical incision and drainage)
Complications of Crohns
Small bowel cancer
Colorectal cancer
Osteoporosis
Perianal fissure/fistula/abscess
Anaemia/malnutrition
Define Ulcerative Colitis
Relapsing and remitting bowel disease that usually involves rectum and can extend up large bowel, up to ileocaecal valve (does not affect anus).
3X more common in non smokers (smoking protective)
Risk factors for UC
- Family History
- HLAB27
- NSAIDs
- Infections
- Not smoking
- Chronic stress/depression
UC Pattern of inflammation, what course does it follow, and how does damage lead to one of its characteristic features
Continuous inflammation affecting only the mucosal layers. Usually starts at rectum, working its way proximally, never past the ileocaecal valve.
Relapsing remitting course (flares with new damage, followed by healing)
Regenerating mucosa forms a scar that looks like polyps (pseudopolyps)
Sign of UC
- LLQ and tenesmus
- Bloody, mucusy diarrhoea
- Abdominal pain, urgency, weight loss and malnutrition
Extraintestinal manifestations
- Uveitis
- Colorectal cancer
- Erythema nodosum
- Pyoderma gangrenosum
- Arthritis
- PSC!
Investigations in UC
Colonoscopy
- Avoid colonoscopy if severe (perforation risk)
- Red, raw mucosa
- Continuous inflammation
- Loss of haustrations
Biopsy
- Pseudopolyps, crypt abscesses, goblet cell depletion, inflammation limited to mucosal layers
What is the most common extra-intestinal manifestation of Crohns and UC
Arthritis
PSC and uveitis more common in UC
Classification of UC
Mild <4 stools/day, small amount of blood
Moderate 4-6 stools/day, varying amounts of blood
Severe 6+ bloody stools/day
Management of UC
Induce remission
Rectal (topical) aminosalicylate - mesalazine/sulfasalazine. Add high dose oral aminosalicylate, and then oral corticosteroid, if extensive or if remission not achieved in 4 weeks.
Maintain remission
Aminosalicylate + azathioprine.
If severe/non responsive,
Colectomy is curative
Complications of UC
PSC
Bowel perforation
Toxic megacolon
Colonic adenocarcinoma
Strictures and bowel obstruction
What is fulminant UC and what is its treatment
Sudden, acute, severe UC flareup
> 10 bowel movements
Continuous bleeding
Abdominal tenderness
Toxicity
Colonic dilation
Managed in hospital
- IV corticosteroid
- IV ciclosporin or infliximab
Consider colectomy
Predisposing factors to UTI in kids
How can constipation cause it
- Infrequent voiding
- Hurried micturition
- Obstruction by full rectum (constipation)
- Poor hygiene
- Neuropathic bladder
- Vesicoureteric reflux - developmental anomaly found in 35% UTI cases
Causative organisms for paediatric UTI
- E coli
- Proteus
- Pseudomonas
Presentation of UTI in babies and older children
Babies/infants non specific
- Poor feeding, irritability, pain, fever, vomiting, urinary frequency
Older children
- Fever, suprapubic pain, vomiting, dysuria, urinary frequency, incontinence, haematuria
- Temp >38 and loin pain suggest upper UTI (pyelonephritis)
Suspect in kids with unexplained fever, vomiting, abdo pain and/or poor feeding
Investigations of UTI
Urine dip - Clean catch best method, but urine collection pads can be used. Avoid other methods. Positive - leukocytes and nitrites.
MSU Culture
Management of UTI in children
- <3 months, refer to paeds
- > 3 months with upper UTI, admit, 7-10 days of antibiotics
- > 3 months lower UTI, 3 days trimethoprim, nitrofurantoin, cefalexin or amoxicillin
How should recurrent and atypical UTI be investigated
Recurrent - abdominal USS <6 weeks
Atypical - Abdo USS during illness
Should also be done for children under 6 months
What is vesicoureteric reflux
Abnormal backflow of urine from bladder into ureter and kidney. Relatively common abnormality of the urinary tract which predisposes to UTIs.
Ureters are displaced laterally, entering at a more perpendicular fashion.
How can VUR present
- Antenatal period scan: Hydronephrosis
- Recurrent childhood UTI
- Reflux nephropathy (chronic pyelonephritis secondary to VUR)
- Renal scarring may produce more renin, causing hypertension
How is VUR diagnosed
Micturating cytourethorgram
May use a DMSA (Dimercaptosuccinic Acid) scan to assess for damage, check for scarring.
Indications for MCUG
Atypical or recurrent UTIs in kids under 6 months
Family history
Dilatation of the ureter on US
VUR
Grading of VUR
1 - Reflux into ureter, no dilation
2 - Reflux into renal pelvis, no dilation
3 - Mild/moderate dilation of the ureter, renal pelvis and calyces
4 - Dilation of the renal pelvis and calyces and moderate ureteral tortuosity
5 - Gross dilation and tortuosity
What is haemolytic uraemic syndrome? What triad is associated with it?
Thrombosis in the small vessels around the body, usually triggered by Shiga toxins, either from E coli 0157 or Shigella. Abx used to treat these also increase risk
Usually follows a gastroenteritis bout and has the classic triad:
- Microangiopathic haemolytic anaemia (destruction of RBC due to thromboses in small vessels causing shearing)
- AKI
- Thrombocytopenia (low platelets)
Presentation of HUS
Diarrhoea is first symptom, turns bloody in 3 days. One week after onset, HUS symptoms start
- Fever
- Abdo pain
- Lethargy and pallor
- Bruising
- Haematuria
- Jaundice
- Confusion
- Reduced urine output
Management of HUS
Supportive treatment
- Hypovolaemia (IV fluids)
- Hypertension
- Severe anaemia and renal failure (blood transfusion and haemodialysis)
What is a paediatric inguinal hernia, what predisposes to it, and how is it treated?
Commoner in males as testis migrate from posterior abdominal wall through inguinal canal, but can occur in females if a patent processus vaginalis.
Children presenting in first few months of life at risk of strangulation
Treated with herniotomy without implantation of mesh.
What are umbilical hernias in children
Relatively common and may be found on newborn exam.
Resolve on their own by 3 years
What are the 3 main complications of hernias
Incarceration - Cannot be reduced back into position
Obstruction - Hernia causes a blockage
Strangulation - Cuts off blood supply causing ischaemia. Surgical emergency.
What is Marasmus
Severe malnutrition caused by an overall deficiency in caloric intake (protein and energy). Usually kids under 5, in developing countries.
Results in extreme fat and muscle loss, causing stunted growth, severe weight loss and immunodeficiency.
Common in areas of famine or severe malnutrition
Presentation of Marasmus
- Severe weight loss
- Emaciation
- Prominent bones, sunken eyes, thin, dry skin
- Irritability
- Growth failure
- Weakness
Treatment of Marasmus
Rehydration
Gradual intro of calories
Micronutrient supplementation
Long term nutritional rehabilitation and infection management
Complications of Marasmus
- Immunodeficiency leading to severe infection
- Electrolyte imbalance (hypokalaemia)
- Hypoglycaemia
- Growth retardation
What is Kwashiorkor
Severe protein malnutrition that occurs despite sufficient caloric intake. Often areas where diet is primarily starchy or carbohydrate heavy with little protein.
Children often appear puffy due to generalised oedema (hypoalbuminaemia), especially in legs and face.
Presentation of Kwashiorkor
- Oedema (legs, face)
- Moon face
- Depigmented hair
- Enlarged, fatty liver
Risk factors for kwashiorkor
Sudden breastfeeding cessation
Poverty
Insufficient protein, high carbohydrate
Food insecurity/scarcity
Complications of kwashiorkor
High mortality if untreated
Heart failure (due to severe oedema)
Sepsis
Severe diarrhoea
Refeeding syndrome if not treated properly
Management of kwashiorkor
Electrolyte and fluid imbalance correction (slow, prevent osmotic demyelination syndrome)
Gradual nutritional rehabilitation, protein rich etc
What is neonatal hepatitis syndrome
Inflammation of liver of newborns usually in first months of life. Can be due to viral infection, genetic disorders, or may be idiopathic.
Inflammation leads to impaired bile flow, damaging liver
Causes of neonatal hepatitis syndrome
Viral (cytomegalovirus, hepatitis B)
Metabolic disease (galactosemia, tyrosinaemia)
Genetic disorders (alpha 1 antitrypsin deficiency)
Family history and prematurity are RF
How does neonatal hepatitis syndrome present
- Jaundice >first 2 weeks, prolonged
- Hepatomegaly
- Dark urine and pale stools
- Poor feeding, failure to thrive
Management of NH Syndrome
- Treatment of underlying cause
- Fat soluble vitamin supplementation (ADEK)
- Ursodeoxycholic acid to aid bile flow
- Liver transplant if severe
What is enuresis?
Majority of children are day and night continent by 3/4. If there is an involuntary discharge of urine by day or night, in a child aged 5 years or older, absent of congenital or acquired defects of the nervous system or urinary tract, this is enuresis.
Primary - Never achieved continence
Secondary - Child has been dry for at least 6 months
Causes of primary enuresis
- Overactive bladder - small volume urination prevents development of bladder capacity
- Fluid intake, (esp. fizzy drinks, juice, caffeine) have diuretic effect
- Failure to wake during deep sleep
- Psychological distress
How is primary enuresis investigated and managed
- 2 week fluid intake, toileting diary
- Try to establish underlying cause
Management
Lifestyle:
- Reduced fluid intake in evenings
- Pass urine before bed
- Positive reinforcement - avoid blame/shame/punishment (star chart for positive behaviours, e.g. going to the toilet before bed)
- Enuresis alarms (requires long term training (>3 months) some may find this a burden/frustrating)
What are some secondary causes of enuresis
- UTI
- Constipation (bowel pressing on bladder)
- T1DM
- New psychological problem (bullying, stress, etc)
- Maltreatment
What is diurnal enuresis
Daytime wetting, dry at night. More frequent in girls.
- Urge incontinence: overactive bladder, little warning before leak
- Stress incontinence: leakage during physical exertion, cough, laugh
Other causes;
- UTI
- Psychosocial
- Constipation
Pharmacological treatment of enuresis
Desmopressin - Vasopressin analogue (anti-diuretic). Reduces volume of urine produced by kidneys, taken at night, for nocturnal enuresis. Can be used shorter term
Oxybutinin - Anticholinergic, reduces contractility of bladder. Helpful in overactive bladder case
Imipramine - Tricyclic antidepressant. Helps somehow
What is Phimosis, what are its causes
The inability for the foreskin to be retracted over the glans. Can be physiological or pathological (caused by scarring or inflammation)
Physiological: Normal, 90% resolve by age 3.
Pathological: Much less common, but caused by infection, scarring, trauma (forceful retraction), chronic inflammation/infections. Fibrosis of the preputial ring prevents retraction. Recurrent infection/inflammation may arise from poor hygiene
Symptoms of phimosis
- Difficulty retracting foreskin
- Painful urination
- Ballooning of foreskin during urination
- Recurrent infections and balanitis
How should Phimosis be investigated and treated
urinalysis, swabs, clinical examination.
Treatment
- Expectant if under 2
- Topical steroids
- Circumcision or preputioplasty if severe or recurrent
- Good hygiene!
Complications of phimosis
Recurrent balanitis
Urinary retention
What is Hypospadias
A condition where the Urethral meatus (opening) is placed on ventral (under) side of the penis, towards scrotum. Also epispadias, where the meatus is displaced to dorsal (top) side of the penis
Significant risk factor: Family history
What is hypospadias normally characterised by
- Ventral urethral meatus
- Hooded prepuce
- Chordee (head of penis bends down - ventrally)
- Urethral meatus may open more proximally (Can be subcoronal, midshaft or penoscrotal)
What is hypospadias sometimes associated with?
- Cryptorchidism (undescended testes)
- Inguinal hernia
Management of hypospadias
May not need treatment if very distal.
Surgery normally after 4 months (usually 12 months of age). Essential child NOT circumcised before surgery, as foreskin can be used in procedure.
Surgery aims to correct meatus position and straighten penis
What is Wilms’ tumour
Wilms’ nephroblastoma, one of the most common childhood malignancies (typically presents under 5). Kidney tumour
What is Wilms’ tumour associated with
- Beckwith-Wiedemann syndrome
- WAGR syndrome (Wilms, Aniridia, Genitourinary malformations, mental Retardation)
- hemihypertrophy
- loss of function mutation on WT1 gene on C11
Features of Wilms tumours
Child under 5 presenting with abdomen mass.
- Painless haematuria
- Flank pain
- Unilateral
- HTN
- Lethargy, weight loss
- 20% metastasise to lung
How should a Wilms tumour be investigated and managed
- Unexplained enlarged abdo mass = arrange paediatric review within 48 hours
- USS to visualise kidneys, CT/MRI for staging, biopsy for definitive diagnosis
- Managed with nephrectomy of affected kidney
- Chemo and radio may be needed
- Prognosis good - 80% cure
What is Nephritic syndrome, what how does it presents and what are its most common causes in kids
Inflammation of nephrons of kidneys.
Presents with:
- Haematuria
- Oliguria (low urine)
- Hypertension (Na+ retention)
- Oedema
- Slight proteinuria BUT <3.5g of protein in 24hrs (Anymore = nephrotic syndrome)
Most common causes of nephritis in kids are post-strep glomerulonephritis and IgA nephropathy
What is Post strep glomerulonephritis
Kidney inflammation that occurs 1-3 weeks after a B haemolytic strep infection (e.g. tonsilitis caused by Strep pyogenes, or skin infection). IgG/IgM/C3 Immune complexes deposit in glomerular basement membrane causing activation of complement and inflammation
Type 3 hypersensitivity.
Strep pyogenes is beta haemolytic, group A
Presentation of post strep glomerulonephritis
Recent (1-3 weeks) tonsilitis, or skin infection (6 weeks)
Proteinura and microscopic Haematuria
Oliguria
Peripheral oedema
Hypertension
Signs of recent infection/sore throat history!
Investigations of PSG
Throat swab & culture - Strep pyogenes
Anti-streptolysin O titre - increased
Low C3
Renal biopsy results:
- Acute, diffuse, proliferative glomerulonephritis
- Endothelial proliferation with neutrophils
- Immunofluorescence: “Starry sky” appearance caused by IgG, IgM, C3 deposits
- Electron microscopy: subepithelial humps caused by lumpy immune complex deposits
Differences between PSG and IgA nephropathy
PSG
- 1-2 weeks post URTI
- Proteinuria>haematuria (which is microscopic)
- Low complement
IgA
- 1-2 days post URTI
- Young males
- Macroscopic haematuria
Management and complications of PSG
No specific
(Penicillin for underlying strep, Furosemide for oedema/HTN, use ACEi if very severe)
- Pulmonary oedema
- Hypertensive encephalopathy
- Severe AKI
Define IgA nephropathy, and what conditions is it associated with
AKA Berger’s disease. IgA deposition in mesangium of kidney (Bowman’s capsule)
Commonest cause of glomerulonephritis worldwide. Presents as macroscopic haematuria in young people (teenagers, YA).
Associated with Henoch-Schonlein Purpura (IgA vasculitis), coeliac, alcoholic cirrhosis
Type 3 hypersensitivity (antigen-antibody complex deposition)
Presentation of IgA nephropathy
Young male, recurrent macroscopic haematuria, starting 1-2 days post recent URTI.
Management of IgA nephropathy
Isolated haematuria, or minimal proteinuria <1000mg/day - no treatment, just follow up.
ACE inhibitors main treatment
Corticosteroids if failure to respond or low eGFR.
Investigations of IgA nephropathy
Urinalysis - Macroscopic haematuria
C3/C4 - NORMAL
Renal biopsy
- Immunoflourescence: IgA and C3
- Histology: Mesangial proliferation, immune complexes
Complications of IgA nephropathy
Worse than post-strep
25% develop end stage renal failure
High proteinuria (>2g/day), hypertension, smoking mark poor prognosis
Brief overview of goodpastures, what type of hypersensitivity is it
Type 2 hypersensitivity
AKA Anti-GBM (Glomerular Basement membrane) disease, autoantibodies attack type 4 collagen in glomerular and alveolar membrane causing haematuria and haemoptysis (bloody cough first)
Anti-GBM and p-ANCA positive
Treated with corticosteroids
What is nephrotic syndrome
Glomerular BM is damaged and becomes permeable to protein.
Triad
- Proteinuria (>3.5g/24hrs)
- Hypoalbuminaemia (lost in urine)
- Peripheral oedema (loss of oncotic pressure)
Other 4 features of nephrotic syndrome
Hyperlipidaemia (causing frothy urine)
Hypercoagulability (frothy urine)
Hypertension
Susceptibility to infection (Loss of Ig in urine)
Most common causes of nephrotic syndrome
Most common cause in children: Minimal change disease.
Also due to intrinsic kidney disease:
- Focal segmental glomerulosclerosis
- Membranoproliferative glomerulonephropathy
What is minimal change disease
Most common nephrotic in children, usually presents before 8. Aetiology not usually clear.
Associated with Hodgkins lymphoma, leukaemia and NSAID use.
Causes oedema, proteinuria and low albumin, in a usually otherwise well child.
How is minimal change disease investigated
Urinalysis - Small molecular weight proteins and hyaline casts
Renal biopsy
- Light microscopy - normal
- Electron microscopy - fusion of podocytes and effacement of foot processes
Minimal change disease management
Oral corticosteroids
1/3 have just 1 episode
1/3 have infrequent relapse
1/3 have frequent relapse which stop before adulthood.
Risk factors for pyelonephritis
Female
Structural urological abnormalities
Vesico-ureteric reflux
Diabetes
What is pyelonephritis and whats its causes
Inflammation of kidney from bacterial infection. Affects renal pelvis and parenchyma. Usually ascending.
Main cause is E Coli (gram negative, anaerobic rod)
Klebsiella
Enterococcus
E coli
Pseudomonas
Staph aureus
Main symptoms of pyelonephritis
Triad
- Fever
- Loin/back pain
- Pyuria (WBC/pus in urine)
Otherwise;
- Dysuria
- Frequency
- Suprapubic discomfort
- Systemic illness
- Renal angle tenderness on exam
- Haematuria
