Paediatrics Flashcards
Causes of pneumonia in children
Commonly viral: adenovirus, rhinovirus, influenza, RSV, parainfluenza
2nd most common= Streptococcus pneumoniae
Presentation of pneumonia in children
Temperature (over 38.5), rapid breathing/difficulty breathing
May have cough, vomiting, chest/abdo pain, decreased activity, loss of appetite/poor feeding
Management of paediatric pneumonia
Antibiotics if indicated. CXR and bloods, sputum culture. Supportive therapy.
What is croup and its presentation
Infection of upper airway (often parainfluenza virus) leads to obstruction of breathing and a barking cough
Px: originally begins with typical cold then 3-5 days of loud barking cough, fever, hoarse voice, noisy/laboured breathing. Symptoms worse at night
How to manage croup?
Who most commonly affected?
Manage at home with comfort measures
Common between 6 months and 3 years
Presentation of asthma?
Cough (nocturnal and worsens with virus), SOB, wheeze/whistling, chest congestion or tightness
Diagnosis of asthma in children?
If over 5: spirometry, PEF, allergy testing
If under 5: need to go off the history
If under 3: may use wait and see approach
How to identify a viral induced wheeze?
No wheeze with exercise, no wheeze except when a/w viral infections
How to manage a viral induced wheeze?
Reliever inhaler for the minority=SABA=salbutamol
If SABA 2-10 puffs PRN to max 4 hourly via a spacer
Otherwise, supportive/comfort care
Natural history of bronchiolitis?
Viral infection of bronchioles, commonly by RSV
Usually affects under 2s
Mainly in the winter and spring months
There is excess mucus, inflammation, constriction and oedema
Presentation of bronchiolitis?
Symptoms increase over 2-5 days: low grade fever, nasal congestion, rhinorrhoea, cough, feeding difficulty
Usually lasts 7-10 days
Investigations for bronchiolitis?
Nasopharyngeal aspirate or throat swab
RSV rapid testing and viral cultures
FBC
Home or hospital for bronchiolitis?
Hospital if severe apnoea/resp distress: grunting, RR>70, central cyanosis, sats<92%
Consider hospital if rr>70, inadequate fluids, clinical dehydration
No role for antibiotics, steroids or bronchodilators
Pathaphysiology of cystic fibrosis
Autosomal recessive mutation in CFTR (CF transmembrane conductane regulator gene)
DeltaF508 is commonest mutation
MUCUS
Reduced airway surface liquid impedes mucus clearance allowing excessive bacterial growth
Pancreas: duct occluded in utero leading to pancreatic insufficiency (can lead to CF-related DM)
GI: increased mucus can cause meconium ileus
Biliary tree: can have cholestasis leading to neonatal jaundice
Neonatal test to identify CF
Heel prick- Guthrie test
Investigations for CF:
Diagnosis= history and positive chloride sweat test
Sweat chloride over 60mmol/L suggestive, 40-60 is borderline
CXR, sweat test, glucose tolerance test, microbiology, LFT, coag, bone profile, PFTs- all generally involved in annual assessments
Heel prick, then genetic testing, then sweat test
Why are children more at risk of epiglottitis than adults?
Epiglottis is floppier, broader, longer and angled more obliquely to trachea and have a larger tongue
Therefore higher risk of acute airway obstruction
Aetiology of epiglottitis?
Reduced since the Hib vaccination
Normally Haemophilus infleunzae and Streptococcus pneumoniae which locally invade
V rarely due to trauma or non-infectious causes
Presentation of epiglottitis
4Ds
Dyspnoea, dysphagia, drooling, dysphonia (muffled hot potato voice)
Typically no cough
Symptoms in less than 12 hours
Stridor is a late sign
Tripod position- leans forward on outstretched arms with neck extended and tongue out
Management of epiglottitis
Secure airway before further investigation
Throat swabs, bloods, lateral neck Xray (thumb print sign, thickened aryepiglottic folds, increased opacity of larynx and vocal cords)
Oxygen, nebulised adrenaline, IV antibiotics (3rd gen cephalosporins eg ceftriaxone), IV steroids, nil by mouth until airway improved
What is acute otitis media
Symptoms
Severe pain and visible inflammation of tympanic membrane lasting days-weeks
Sxs: pain, malaise, fever, coryzal symptoms
What causes otitis media
Infection from nasopharyngeal organisms migrating via eustachian tube (which is shorter wider and straighter in children)
Bacterial= S.pneumoniae
Viral=RSV, rhinovirus
Investigations for otitis media
ALWAYS test function of facial nerve on examination
Otoscopy
Discharge sent for microscopy and culture
How to manage acute otitis media
Watch and wait- most spontaneously resolve within 24 hours
Simple analgesia
Complication monitoring= mastoiditis which will need IV abx
What proportion of children will have had at least 1 episode of otitis media by age 3
more than 2/3
What is otitis media with effusion
glue ear
Viscous inflammatory fluid build up leading to conductive hearing impairment via chronic inflammatory changes and eustachian tube dysfunction
What are risk factors for otitis media with effusion?
Bottle fed, paternal smoking, atopy, genetic disorders egCF and Downs
Presentation of otitis media with effusion?
Difficulty hearing and pressure sensation
O/E: TM will be dull and light reflex is lost, may see a bubble behind the TM
How to manage otitis media with effusion?
Active surveillance- around 50% resolve within 3 months
If no resolution may need hearing aids or myringotomy and grommet insertion
Indications for grommets?
Small plastic tubes inserted in opening in eardrum
Relieve OME if hearing loss is 25-30dB on 2 occasions 3 months apart
Causes of deafness in children (infections)
Temporary=OME
Meningitis, mumps and measles can cause it
Aetiology of tonsillitis
Viral mostly
Bacterial-group A strep most common (S.pyogenes)
Most common in age 5-15
Tonsillitis- bacterial vs viral assessment
Centor score:
Age, exudate, tender/swollen anterior cervical LNs, temp over 38, absent cough
Management of tonsillitis
Viral is self limiting
Bacterial- should swab for culture and commence antibiotics (usually benzylpencillin)
What is the epidemiology of periorbital cellulitis?
Peak in under 10s
Males twice as commonly affected
Peak occurrence in later winter and early spring
What are the causes of periorbital cellulitis?
Commonly from contiguous spread from sinuses with ethmoidal sinus being most common
Others= dental infection, trauma, foreign bodies, impetigo
S. pneumoniae and S. aureus
What is the classification used for periorbital cellulitis?
Chandler classification, types i-v
What is type I of chandler classification?
Pre-septal cellulitis- inflammation and oedema anterior to orbital septum
type II of chandler classification?
post-septal, orbital cellulitis:
inflammation into orbital tissue but no abscess formation
Eyelids may be swollen and may have conjunctival chemosis with variable degree of proptosis and visual loss
type III chandler classification?
subperiosteal abscess
Pronounced eyelid oedema, conjunctival chemosis and tenderness along orbital rim with variable degree of motility/proptosis/visual acuity changes
type iv chandler classification?
intra-orbital abscess-pus inside or outside muscle cone
Proptosis, conjunctival chemosis, decreased motility and visual loss can be severe
type v chandler classification?
Cavernous sinus thrombosis
Bilateral marked eyelid oedema and involvement of CNs III, V, VI
May have sepsis signs
How to manage periorbital cellulitis?
If mild preseptal can do from home with broad spectrum empirical abx
If more extensive- hospital, IV abx, supportive therapy
If large abscesses, intracranial complications at px and in frontal sinusitis, need urgent drainage
Complications of periorbital cellulitis?
Visual related (vision loss is 11%)- start to lose red colour vision (sign of optic nerve compromise) Neurological complications eg sepsis, intracranial abscess, cavernous sinus thrombosis
Describe the types of manifest and latent strabismus
Manifest: Esotropia=eye turns in Exotropia=eye turns out Hypertropia=eye goes up Hypotropia=eye goes down
Latent: Esophoria=inwards under occluder Exophoria=outwards under occluder Hyperphoria=up under occluder Hypophoria= down under occluder
what tests to identify strabismus
Cover test (manifest squint) Cover/uncover test (latent squint)
What are potential septal defects?
Ventricular septal defect
Atrial septal defect
Atrioventricular septal defect
Tetralogy of fallot
What is the most common septal defect?
VSD
What is eisenmenger’s syndrome?
A left to right shunt switches direction due to an increase in pressure of the low oxygen-containing side
CYANOTIC
Sizes of VSD and what it may cause
Small=restrictive VSD-generally asx
Moderate=dilatation of left atrium and ventricle, at risk of congestive heart failure and arrhythmias
Large=early HF and severe pulmonary hypertension, may develop eisenmenger’s syndrome
What presentation with VSD?
tachypnoea
SOB, feeding problems, developmental issues
undernourished, forehead sweat, increased breathing work, cyanotic, may have down’s syndrome, clubbing, hyperactive precordium, thrill, systolic murmur
Murmur for VSD and where to listen?
Systolic murmur (holosystolic, pansystolic or early systolic) Best heard over tricuspid area (lower left sternal border) with radiation to right lower sternal border
Gold standard diagnosis of septal defect?
Echo
Management of VSD?
Qp/Qs (pulmonary to systemic blood flow ratio) of at least 2.0 requires surgical repair
Aim to close large VSDs within first 2 years of life
75% of small VSDs close spontaneously by age 10
What is the second most common septal defect?
ASD
What are the 5 types of atrial septal defect?
patent foramen ovale (most common) ostium secundum defet ostium primum defect sinus venosus defect coronary sinus defect (least common)
Presentation of ASD?
Vast majority are asymptomatic
Large= tachypnoea, poor weight gain, recurrent chest infections
Type of murmur in ASD, and where to auscultate?
Soft systolic ejection murmur
Best heard in pulmonary area (left upper sternal edge)
RFs for ASD
maternal smoking in first trimester, maternal diabetes, maternal rubella, maternal drug use eg cocaine/alcohol
Management of ASD?
If less than 5mm, spontaneous closure within 12 months
Surgical closure required if bigger than 1cm
What group of people is more likely to have an atrioventricular septal defect?
People with Down’s syndrome
Describe the pathaphysiology of an AVSD?
2 types
Failure of endocardial cushions to fuse properly
Complete AVSD: large left to right shunt causes excessive pulmonary blood flow leading to HF and pulmonary vascular resistance
Partial AVSD: left to right shunt and volume overload of RA and RV but the pulmonary artery pressure is only mildly elevated
Presentation of AVSD?
Tachypnoea, tachycardic, poor feeding, sweating, failure to thrive, all with a complete AVSD are symptomatic by 1 year
Prominent sternal heave, murmur
Murmur in AVSD and where to auscultate?
Ejection systolic murmur in pulmonary area (left upper sternal border)
Mid-diastolic murmur over tricuspid area (left lower sternal border)
Holosystolic murmur might be heard at apex
Management of AVSD?
Surgical management as patients would die by age 2-3 without
Describe the tetralogy of fallot?
Ventricular septal defect
Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta
Most common cyanotic congenital heart disease
Presentation of tetralogy of fallot?
Mild=usually asx, but as disease progresses, develops cyanosis
Mod-severe=cyanotic and resp distress
Extreme= pulmonary atresia or absent pulmonary valves
Murmur
If tetralogy of fallot isn’t managed, what can occur?
polycythaemia, cerebral abscess, stroke, infective endocarditis, HF, death
Murmur in tetralogy of fallot and where to listen?
Pansystolic murmur best heard over mid or upper left sternal edge
Smaller the VSD=louder the murmur
What are tet spells?
Hypoxic spells in tetralogy of fallot with peak incidence between 2-4 months of life:
Paroxysm of hyperpnoea, irritability, increasing cyanosis
Management of tetralogy of fallot?
Medical= squatting, prostaglandin infusion, beta blockers, morphine, saline bolus
Surgical management for definitive repair or palliative management
Which defects have a higher risk of HF?
Significant left to right shunt= VSD, ASD, PDA
Transposition of the great arteries
Describe the two types of transposition of the great arteries?
dextro-transposition=aorta is anterior and to the right of the pulmonary artery
levo-TGA= aorta anterior and to the left of the pulmonary artery
When do you get cyanotic heart disease?
Absent/not wide enough tricuspid/pulmonary/aortic valve Coarctation of aorta Ebstein anomaly Tetralogy of fallot TGA Truncus arteriosus
Anywhere with a right to left shunt
What is rheumatic fever?
Systemic illness 2-4 weeks after pharyngitis due to cross reactivity to group A beta-haemolytic streptococcus (S. pyogenes)
What are the diagnostic criteria for rheumatic fever? Describe
Revised Jones diagnostic criteria= positive throat culture or anti-streptolysin O/anti-DNA B titre and 2 major or 1 major+2 minor criteria
What are the major criteria in the revised jones criteria?
SPECS
Sydenham’s chorea, polyarthritis, erythema marginatum, carditis, subcut nodules
What are the minor criteria in the revised jones criteria?
CAPE CRP/ESR raised Arthralgia Pyrexia ECG-prolonged PR
Management of acute rheumatic fever?
Antibiotics=benzathine benzylpenicillin
Aspirin/NSAIDs
Assess for emergency valve replacement
Secondary prevention with abx every 3-4 weeks
What factors lead to subacute bacterial endocariditis?
Endothelial damage, platelet adhesion, microbial adherence
What organisms cause IE?
S. aureus, S. viridans, S. pneumoniae, HACEK organisms
Presentation of IE?
persistent low grade fever, heart murmur, splenomegaly, petechiae/oslers nodes/janeway lesions/splinter haemorrhages
Criteria for diagnosis of IE? describe
Modified Dukes criteria
2 major
1 major and 3 minor
5 minor
Major criteria in modified dukes?
positive blood cultures, evidence of endocardial involvement (echo)
Minor criteria in modified dukes?
predisposition, fever, vascular phenomena, immunologic phenomena, microbiology evidence, other echo findings
Management of IE?
IV antibiotics
May require surgical intervention
What factors aid reflux in GORD?
Low tone of muscular portion of lower oesophagus, short and narrow oesophagus, delayed gastric emptying, shorter lower oesophageal sphincter, liquid diet and high calorie requirement (distends stomach), significant periods recumbent
Presentation of GORD?
Distressed, unexplained feeding difficulties, hoarseness +/- chronic cough, single episode pneumonia, faltering growth, if able may report pain
When does GORD or regurgitation (=posseting) present normally
Within 2 weeks of life
How to manage a breast fed baby with gord
Use alginate eg gaviscon mixed with water after feeds
How to manage a formula fed baby with gord
1= do not over feed (less than 150ml/kg/day) 2= decreased feed volume by increasing frequency (2-3 hourly) 3= use feed thickener 4= stop thickener and use alginate added to formula
What to use after alginate if no success in GORD?
histamine antagonists or ppi
Name 5 potential causes of poor feeding
Infection, metabolic disorders, genetic disorders, structural abnormalities, neurological disorders, mum and feeding technique
Presentation of pyloric stenosis
Baby starts bringing up small amounts of milk after feeding, which gets worse over a few days
Then projectile yellow vomit (milk curdles in stomach acid)
Reduced faeces
If untreated: dehydration and not gaining weight
When does pyloric stenosis present
About 6 weeks after birth
How to manage pyloric stenosis
Pyloromyotomy (laparoscopically)
Potential presentation of IBS
Bloating, cramping, chronic or intermittent diarrhoea/constipation, urgency with defecation, incomplete sensation of defecation, passage of mucus in stool
May have dizziness, nausea, loss of appetite
How to manage IBS
Diet and lifestyle management eg small meals, more often, low fat and high carbs
How common is gastroenteritis?
Very!
At least one episode per year for most children
Common causes of gastroenteritis
Rotavirus is most common infantile (NB rotarix oral vaccine at 8 and 12 weeks)
Norovirus commonest acorss all age groups
Campylobacter is most common bacterial cause
Also E.coli and adenovirus
Presentation of gastroenteritis
Sudden onset diarrhoea +/- vomiting, abdo pain, mild fever
bloody diarrhoea if campylobacter- generally due to undercooked meat and unpasteurised milk
Management of gastroenteritis
Self limiting
Send a stool sample if ?septicaemia/blood or mucus in stool/immunocompromised
Encourage fluids (not carbonated drinks)
IV or oral therapy if dehydrated
Give full strength milk asap
Leave it 48 hours before returning to school
How long do sxs of gastroenteritis last?
Diarrhoea around 5-7 days
Vomiting around 1-2 days
Potential complications of gastroenteritis?
Haemolytic uraemic syndrome, reactive complications eg Reiter’s syndrome, toxic megacolon, acquired/secondary lactose intolerance
What counts as constipation in children?
Less than 3 complete stools/week
Hard/large stool
Rabbit droppings
Overflow soiling in children over 1
Management of simple constipation in children?
Diet and lifestyle advice- high fibre, increase fluids
Then add Macrogol eg Movicol
Then add stimulant laxative eg Senna
Consider adding in lactulose
In secondary care can do manual evacuation, antegrade colonic enema, polyethylene glycol solutions
Describe the pathophysiology of appendicitis
Luminal obstruction (faecolith or lymphoid hyperplasia, impacted stool) Decreased venous drainage and localised inflammation by commensal bacteria causes and increased pressure. This causes ischaemia which can lead to necrosis and perforation
Presentation of appendicitis
Dull peri-umbilical pain transfers to a sharp right iliac fossa pain
May have vomiting, anorexia, nausea, diarrhoea or constipation
MAGNET: migration of pain to RIF, anorexia, guarding, nausea, elevated temp, tenderness in RIF
What can be found on examination of appendicitis
Rebound tenderness and percussion pain over McBurneys point and guarding Psoas sign (pain when patient lies on left side while right thigh is flexed backward) Rovsing's sign (pain in RLQ upon palpation of LLQ)
Management of acute appendicitis
Antibiotics and laparoscopic appendicectomy
Why are children at higher risk of appendix perforation?
Tend to have delayed presentation
Two types of childhood hernia?
Umbilical and epigastric
How to manage hernias in children?
Umbilical- surgery if still present after 3 years
Epigastric- surgery only if uncomfortable or a nuisance
Describe how Crohn’s disease affects the body (microbiology and location)
Remitting and relapsing disease, affects anywhere between the mouth and the anus
Transmural inflammation, deep ulcers and fissures with cobblestone mucosa, skip lesions
Non-caseating granulomatous inflammation
Presentation of Crohns disease
Abdominal pain and bloody/mucus containing diarrhoea
Oral aphthous ulcers, perianal disease (tags, fistulae)
Extra-intestinal features=MSK, eyes, skin, renal, hepatobiliary
Investigations for IBD?
Faecal calprotectin, routine bloods, stool microscopy and culture, colonoscopy with biopsy (=definitive diagnosis and can distinguish between UC and CD)
How to manage Crohn’s disease? Acute attack
In acute attack: avoid anti-motility drugs eg loperamide as it can cause toxic megacolon
In acute attack: fluid resus, nutritional support, prophylactic heparin, corticosteroid and immunosuppressant eg mesalazine or azathioprine
How to manage Crohn’s disease? Maintenance
Azathioprine, smoking cessation
Surgery can be used- ileocaecal resection, stricturoplasty, small/large bowel resections
Course and pathophysiology of ulcerative colitis?
Relapsing/remitting
Diffuse continual mucosal inflammation of large bowel only. Begins in the rectum and spreads proximally.
Mucosal and submucosal inflammation only, crypt abscesses and goblet cell hypoplasia
What is a protective factor against UC?
Smoking!
Presentation of UC?
Bloody diarrhoea, PR bleeding and mucus discharge, increased faecal frequency, urgency and tenesmus
Extra-intestinal: MSK, skin, eyes, hepatobiliary
How to induce remission in UC?
Corticosteroid and immunosuppressant eg mesalazine or azathioprine
Maintenance management of UC?
Immunomodulators- mesalazine or sulfasalazine
Next line=infliximab
Surgery= ileostomy and complete resection= curative, but many have sub-total colectomy to begin with
Pathophysiology of Coeliac disease
T cell mediated autoimmune response.
Response to gliadin and genetic (HLA-DQ2/DQ8)
Anti-gluten CD4 t cell response, with anti-gluten antibodies, anti-tissue transglutaminase, endomysin antibodies and intraepithelial lymphocytes activated
Causes epithelial cell destruction and villous atrophy
What are the forms of coeliac disease?
Classical, atypical, latent, silent, potential
Describe the classical form of coeliac disease
Arises between 9 and 24 months
Crypt hyperplasia and villous atrophy
Features of malabsorption eg steatorrhoea, failure to thrive, anorexia
Describe the atypical form of coeliac disease?
No intestinal symptoms
Extra-intestinal symptoms eg osteoporosis, anaemia, peripheral neuropathy
Describe the latent form of coeliac disease
HLA DQ2/8 positive but normal mucosa present
Describe the silent form of coeliac disease
Damaged mucosa, positive serology, no symptoms
What is potential coeliac disease
Genetically predisposed people
What are potential extra-intestinal features of coeliac disease?
Dermatitis herpetiformis, osteoporosis, short, delayed puberty, arthritis
How to investigate for coeliac disease?
Serology
Must have had gluten in diet for at least 6 weeks prior to testing
IgA and IgAtTG, IgAEMA
Duodenal biopsy
Common causes of failure to thrive
Down syndrome, cerebral palsy, heart disease, infections, milk allergy, CF, coeliac disease, GORD
What is marasmus
Protein and energy malnutrition.
Loss of fat mass especially buttocks
Chronic diarrhoea and emaciated body type
There is NO oedema
What is marasmus usually a sign of?
Poor socioeconomic status, child abuse+neglect, lack of food resources
How to manage marasmus?
Similarly to kwashiorkor and reintroduce food slowly and correct any electrolyte imbalances
What is kwashiorkor
Inadequate protein with a reasonable calorie intake
Presentation of kwashiorkor
Fatigue, irritability, lethargy, failure to thrive, loss of muscle mass, generalised oedema, pot belly, fatty liver, prone to infections, hair and skin changes (erythematous, exfoliation, pigment, dry hair, thin nails)
What is seen in bloods for kwashiorkor?
low glucose, low protein, high cortisol and GH, low salt, iron deficiency anaemia, metabolic acidosis
Management of kwashiorkor?
Correct fluids/electrolytes- needs to be done in about 48 hours
Reintroduce foods slowly afterwards using RUTF (ready to use therapeutic foods eg peanut butter, milk powder, sugar, vegetable oil, minerals and vitamins)
Treatment takes around 2-6 weeks
Pathophysiology of Hirschprung’s disease
Congenital aganglionic megacolon disease Aganglionic segment (short/long/total) remains in tonic state so there is a failure in peristalsis Functional obstruction due to accumulated faeces
Presentation of Hirschprung’s disease
Abdominal distension, bilious vomiting, failure to pass meconium within 48 hours after birth
Males 4 times more likely
What is a big complication of Hirschprung’s disease
Hirschprung’s enterocolitis: stasis allows bacterial proliferation, which can cause sepsis and death
How to investigate for Hirschprungs
Initially a plain X ray
Gold standard=rectal suction biopsy
Management of Hirschprungs
IV antibiotics, NG tube, bowel decompression initially
Definitive= surgery to resect aganglionic section and connect unaffected bowel to dentate line
What is intussusception?
Who does it happen to?
Movement/telescoping of one part of bowel into another
Peak at 5-7 months, rare after 2 years, males twice as likely
Most cases are ileo-colic type (distal ileum passes into caecum through ileo-caecal valve)
Presentation of intussusception
Sudden onset, inconsolable crying episodes, pallor, child may draw knees up to chest
Later stages= red-currant consistency stools (blood and mucus), sausage shaped mass in RUQ
How to diagnose intussusception?
abdominal uss
How to manage intussusception?
Nonoperative=air or contrast enema
Surgical reduction if contraindication to enema
What is meckel diverticulum?
Outpouching of lower part of small intestine- a remnant of umbilical cord
Presentation of meckel diverticulum?
Most asx
In minority: pouch releases acid causing bleeding ulcers
In serious complications, can lead to peritonitis
Pouch can twist causing volvulus or intussusception
What is malrotation with volvulus?
Occurs during embryonic development at about the 10th week
Bowel twists so blood supply is cut off
Presentation of malrotation with volvulus?
Bouts of crying, pull legs into body, not passing faeces, cramps, green vomit, may become dehydrated
Most diagnosed by age of 1
How to manage malrotation with volvulus
Dx by Xray
Tx= operation- stop necrosis of bowel and may need bowel resection
What is toddler diarrhoea
Chronic nonspecific diarrhoea, at least 3 loose watery stools/day
Some alternate with constipation
Self limiting at about age 5-6
NOT due to malabsorption or serious bowel problem
What are the 4Fs for diet consideration in toddler diarrhoea
Fat, fluid, fruit juices and fibre
What is colic
Self-limiting repeated episodes of excessive and inconsolable crying for at least 3hrs a day, at least 3 days a week for at least 1 week
Occurs in up to 4 months of age
Presentation of colic
Often crying in late afternoon or evening, draws knees up and arches back
Management of colic?
REASSURANCE
Soothing strategies, parental wellbeing aids
Discourage simeticone (infracol) or lactase drops, probiotics, herbal supplements and maternal diet modification
Exclude other possible causes of distress
Describe the three types of biliary atresia
Type I= common bile duct atresia with patent proximal ducts
Type II= common hepatic duct atresia with cystic structures in porta hepatis
Type III= R and L hepatic duct atresia to level of porta hepatis (most common)
What is biliary atresia?
Extrahepatic bile ducts obliterated by inflammation and subsequent fibrosis
Presentation of biliary atresia?
Presentation shortly after birth
Persistent jaundice
Pale stools and dark urine in term infants with normal birth weights
What investigations are required in biliary atresia and what results shown?
LFTs: increased GGT, TGs normal
USS
Liver histology from percutaneous biopsy
Management of biliary atresia?
Surgery, liver transplant needed before 8 weeks
Prognosis without treatment is 18 months
Complications of biliary atresia?
Ascending cholangitis, cirrhosis, portal hypertension, liver failure, hepatocellular carcinoma
Pathophysiology of cow’s milk protein allergy
Immune-mediated allergic response to casein and whey
IgE mediated type 1 hypersensitivity or non-IgE(T cell activation)
Presentation of cow’s milk protein allergy
Acute and rapid onset (less than 2 hrs after ingestion), pruritus, erythema, acute urticaria, angio-oedema, oral pruritus, colicky abdo pain, vomiting, diarrhoea, may have resp signs
Management of cow’s milk protein allergy
Elimination diet for at least 6 months or until 9-12 months old, then reevaluate every 6-12 months
Replace formula with hypoallergenic: extensively hydrolysed or amino acid formula
What is wilson’s disease?
Autosomal recessive disorder of hepatic copper deposition
Mutations in gene ATP7B
Typical onset in 2nd-3rd decade
Presentation of wilson’s disease
HEPATIC features: acute liver failure, chronic hepatitis and cirrhosis, fulminant hepatic failure+/- haemolytic anaemia
PSYCH features: severe depression, neuroses
NEURO features: asymmetrical tremor, speaking difficulty, hypersalivation, clumsiness with hands
Kayser-fleischer rings, sunflower cataracts
May be a/w rheumatoid-osteopenia, cardiac arrhythmias, hypoparathyroidism, pamcreatitis, infertility
What investigations for wilson’s disease
Low serum caeruloplasmin
Increased urinary copper
Liver biopsy
Assay
Management of wilson’s disease
Avoid high copper food, penicillamine or zinc/trientine (chelating agents), may need liver transplant
Choledochal cysts pathophysiology?
Usually in extrahepatic ducts (CBD and HDs), sometimes in intrahepatic ducts
Type I cysts are most common-lower end of CBD joins up with pancreatic duct: pancreatic juice and bile mix leading to a weakening of bile duct wall and ballooning- can lead to pancreatitis and cholangitis
Management of choledochal cyst
USS to identify
Commonly incidental finding
Surgery for removal of cyst
Presentation of choledochal cyst
May have jaundice, intermittent abdo pain, cholangitis, peritonitis, lump, pancreatitis
Aetiology of neonatal hepatitis syndrome
in 20%- viral (cytomegalovirus, rubella, hep a/b/c)
80%- nonspecific virus
Presentation of neonatal hepatitis syndrome
Usually at 1-2 months
Jaundice, not gaining wt/ht, hepatosplenomegaly
Management of neonatal hepatitis syndrome
Ix: liver biopsy and bloods
Mx: none specific, may need vitamins, potentially a liver transplant
Presentation of liver failure in children
Increased AST and ALT with prolonged clotting +/- jaundice +/- encephalopathy
Causes of liver failure
Viruses eg HSV, EBV, CMV, Hep A/B/E
Inherited metabolic disorders eg Wilsons, mitochondrial
Toxins
Medication eg erythromycin
Autoimmune
Low blood flow eg HF
Chronic: hep C, autoimmune, haemochromatitis, alpha 1 antitrypsin deficiency, CF, biliary atresia, sclerosing cholangitis
Common causes of UTI in children
E coli, Klebsiella, S. saprolyticus
Nb/ pseudomonas suggests structural abnormality
Management of UTI in children
In under 3 months: IV cefuroxime for 7 days and USS renal tract
In over 3 months: oral abx for 3/7 (trimethoprim, nitrofurantoin, cephalosporin, amoxicillin)
If pyelonephritis: 7-10 days abx, ciprofloxacin or co-amoxiclav
Presentation of pyelonephritis
At least 38 degrees and bacteriuria
or
Less than 38 degrees and loin pain/tenderness and bacteriuria
When would you expect children to be dry overnight
Age 5
Factors contributing to nocturnal enuresis
Small bladder, inability to recognise full bladder, hormones, UTI, sleep apnoea, diabetes, chronic constipation, structural problem in urinary tract or nervous system
Management of nocturnal enuresis
<5= reassurance and advice
Non drug treatment: fluid intake, diet, toilet behaviour, rewards system then enuresis alarm if not responding (still more than 1-2 bed wets in a week)
Drug= desmopressin if over 5 +/- enuresis alarm
Imipramine hydrochloride is last line
Causes of acute renal failure
Hypoperfusion (blood loss, surgery, shock)
Blockage of urinary tract
Nephrotoxic drugs
Haemolytic uraemic syndrome (e.coli usually)
Glomerulonephritis
Symptoms of acute renal failure
Haemorrhage, fever, rash, bloody diarrhoea, vomiting, anuria or polyuria, pallor, oedema, eye inflammation, stomach mass
Treatment of acute renal failure?
Treat underlying cause U+Es Dietary changes Antihypertensives Iv fluids Diuretics
How long for it to be classed as chronic renal failure?
Potential causes?
Over 3 months
Causes: long term blockage, alport syndrome, nephrotic syndrome, PKD, cystinosis, chronic conditions eg diabetes, untreated acute kidney disease
What is alport syndrome
Inherited condition- X-linked most common, but can be autosomal recessive/dominant
Deafness, renal damage and eye defects
Treatment of chronic renal failure
Diuretics
Dialysis and transplant
Diet changes (limit protein, potassium, phosphorus, sodium)
Growth promoters/bisphosphonates/iron tablets
What defines nephrotic syndrome
Generalised oedema, heavy proteinuria (>200mg/mmol) and hypoalbuminaemia (<25g/L)
(Flattened podocytes allow protein leakage)
(Normal protein less than 20mg/mmol, normal albumin 35-45g/L)
Causes of nephrotic syndrome
Which is most common?
Minimal change disease=most common
Others= congenital nephrotic syndromes, focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis
Management of nephrotic syndrome
High dose steroids- most respond well but some will have relapse (=steroid-resistant nephrotic syndrome)- in which case may need maintenance low dose steroids or immunomodulatory drugs eg cyclophosphamide
Low salt diet, prophylactic abx (as they leak Igs and steroids!), immunisations
Prednisolone for first episode 60mg/m2 for 4-6 weeks then wean down
What is minimal change disease
Microscopically damage not evident. Idiopathic
Develop nephrotic syndrome more quickly but much more responsive to treatment
What is congenital nephrotic syndrome
What is needed in management
Genetic condition (parents need 1 faulty and 1 healthy gene each to pass on)
Requires frequent albumin infusions to aid growth and development
Typically leads to renal failure
Causes of nephritic syndrome
Post-streptococcal glomerulonephritis, henoch schnolein purpura, alport syndrome, sle/lupus nephritis
Describe post-strep glomerulonephritis
10-14 days after skin/throat infection with group A strep
Haematuria, oedema, decreased urine output, htn, proteinuria, tired
Normally self limiting so needs supportive tx only
If strep infection persisting= penicillin
What is henoch schnolein purpura
IgA vasculitis
Rash of raised red or purple spots usually on legs and bottom (extensor surfaces)
Px of henoch schonlein purpura
Rash, arthritis, abdo pain, kidney impairment (self limiting and mild usually), may get PR bleeding and intussusception
Usually all preceded by 2-3 weeks of fever, headache, arthralgia/myalgia, abdo pain
Mx of Alport syndrome
ACEi/ARB, diuretics, limit sodium
Monitor for potential renal failure
Mx of Lupus nephritis
Immunosuppressants eg cyclophosphamide or hydroxychloroquine, antihypertensives, may need steroids
Types of hypospadias
When does it form
Subcoronal (near head of penis)
Midshaft
Penoscrotal (where penis and scrotum meet)
Weeks 8-14 of pregnancy
Management of hypospadias
Surgery between 3 and 18 months, may need to be done in stages
Types of renal malformation?
Autosomal recessive polycystic kidney disease
Duplication (single renal unit with more than one collecting system)
Fusion (kidneys joined, but ureters enter bladder on each side)- most common= horseshoe
Malrotation (little clinical significance)
Multicystic dysplastic kidney (nonfunctioning renal unit but contralateral kidney normally fine)
Renal agenesis (bilat=fatal, unilateral)
Renal ectopia=kidney fails to ascend from true pelvis
Renal hypoplasia (underdeveloped)
Pathophysiology of vescioureteral reflux
Urine flows from bladder back into ureters, potentially causing infection
Shorter than normal attachment between ureter and bladder and incompetent valve
Describe the different grades of vesicoureteral reflux
I=into ureter
II=into ureter and renal pelvis without distension
III=into ureter and renal pelvis with mild swelling
IV= mod swelling
V=severe swelling and twisting or ureter
Management of vesicoureteral reflux
Can go away by itself (junction gets longer with age)
Encourage frequent voiding
May need surgery to stop reflux
Triad of haemolytic uraemic syndrome
Coombs test negative, thrombocytopenia, AKI
Typically a/w e.coli
What is haemolytic uraemic syndrome
Toxins bind from e.coli causing thrombin and fibrin to be deposited in microvasculature
RBCs damaged while passing through occluded small vessels causing haemolysis
Px haemolytic uraemic syndrome
Profuse diarrhoea turning bloody 1-3 days later, fever, abdo pain and vomiting
Mx of HUS
Supportive- fluid and electrolyte mx, dialysis PRN, antihypertensive, keep circulating volume up
Where abouts are babies usually affected with atopic dermatitis?
Face, neck, scalp, elbow and knees
Where abouts are children usually affected with atopic dermatitis?
Inside elbows, back of knees, sides of neck, around mouth, wrists, ankles and hands
General management of eczema?
Avoid triggers, keep nails short, emollients
Rest of treatment depends on severity: may include corticosteroids, abx, antihistamines, calcineurin inhibitors, phototherapy, immunomodulators or biologic medications
What steroid would you give for mild eczema?
Hydrocortisone 1%
What steroid for moderate eczema?
Betamethasone valerate 0.025% or clobetasone butyrate 0.05%
What steroid for severe eczema?
Betamethasone valerate 0.1%
May need maintenance steroids or topical calcineurin inhibitors eg tacrolimus
What is Stevens-Johnson syndrome?
Toxic epidermal necrolysis. Rare, acute and potentially fatal skin reaction with sheet-like skin and mucosal loss
Nearly always caused by medications
What medications commonly cause Stevens-Johnson syndrome
Cotrimoxazole, penicillin, cephalosporins, anti-convulsants, allopurinol, paracetamol, NSAIDs
Usually within the first week of abx therapy
Presentation of Stevens-Johnson syndrome
Initially a flu-like illness
Then, tender/painful red skin rash on trunk. extending to face and limbs over a course of hours-days
(Skin lesions: macules/diffuse erythema/targetoid/blisters)
The blisters merge to form sheets of skin detachment
At least 2 mucosal surfaces affected eg conjunctivitis/mouth ulcers/cough/diarrhoea
Acute phase lasts 8-12 days
Management of Stevens Johnson syndrome
Skin biopsy shows keratinocyte necrosis
SCORTEN illness severity score to predict mortality
Cessation of suspected causative drug, admission, analgesia, temp maintenance, nutrition and fluids
General care of affected mucosal surfaces
Classification of allergic rhinitis?
Seasonal (if due to grass and tree pollens=hay fever)
Perennial (throughout year, due to dust mites and animal dander)
Intermittent (less than 4 days/week or less than 4 consecutive weeks)
Persistent (more than 4 days/week or more than 4 consecutive weeks)
Occupational
Management allergic rhinitis? Mild-mod
Nasal irrigation with saline
Allergen avoidance
PRN antihistamine- intranasal as first line eg azelastine
Can use 2nd generation non sedating oral eg loratidine or cetirizine for mild-mod symptoms
What can be used in mod-severe allergic rhinitis?
Intranasal corticosteroid during periods of allergen exposure eg mometasone fluroate or fluticasone propionate
For hayfever, what pollens affect you when?
Tree=early-late spring
Grass=late spring-early summer
Weed=early spring-late autumn
What causes urticaria?
Histamine release
Due to: allergens, bee and wasp stings, autoimmune/idiopathic (chronic), physical triggers eg cold/friction, medications
Types of antihistamine
Non sedating, sedating, short and long acting=H1 antihistamine
H2 antihistamines
What is angio-oedema?
Swelling deep to the skin, commonly soft areas of skin eg eyelids and lips, more painful than weals/hives and takes longer to clear.
Presentation of anaphylaxis
Skin features-urticaria, erythema, flushing, angio-oedema
Involves at least 1 of Resp/CV/GI system
How to manage anaphylaxis
ABCDE, high flow oxygen, IM adrenaline, repeat 3-5 mins if required, treat a drop in BP with fluid bolus, consider salbutamol if wheeze, antihistamines for itch(cetirizine)
Prescribe an epipen
What are the most common vascular birth marks?
Macular stains or salmon patches: Angel’s kisses (forehead/nose/upper lip/eyelids- disappear with age) or Stork Bites (back of neck, disappear with age)
Hemangioma (grow rapidly 6-9 months then shrink and lose red colour)
Port-wine stain/nervus flammeus (grows with the child, often face/arms/legs, flat pink/red/purple mark)
What are the most common pigmented birth marks?
Moles/congenital naevi, cafe au lait spots, mongolian spots (blue-grey spots on lower back or buttocks, common on darker skin)
Causes of infectious rash in children
Meningococcal, steven-johnsons syndrome, impetigo, kawasaki, staphylococcal scalded skin syndrome, eczema herpeticum, erythema nodosum, erythema multiforme, measles, glandular fever, hand foot and mouth disease, erythema infectiosum, chicken pox, nappy rash, scabies, tinea corporis, tinea capitis, mollusucm contagiosum
What are the three phases in Kawasaki disease?
Acute phase=weeks 1-2
Subacute phase= weeks 2-4
Convalescent phase=weeks 4-6
Presentation of kawaski disease in acute phase?
Fever for at least 5 days (over 38), rash, red or hard fingers or toes, hand/foot swelling, conjunctival infection, dry/cracked lips, strawberry tongue, swollen lymph nodes
What happens in subacute phase of kawasaki disease?
Symptoms less severe. Fever subsides, may have diarrhoea, vomiting, abdo pain, pyuria, lethargy, headache, malaise, jaundice and peeling skin
What happens in the convalescent phase of kawasaki disease?
Begin to recover, symptoms start to go away, residual lethargy.
Pathophysiology of kawasaki disease?
Idiopathic. Not contagious.
Also known as mucocutaneous lymph node syndrome.
Mainly affects under 5s.
What are NICE criteria for diagnosis of kawasaki?
Over 38 degrees for at least 5 days and at least 4 key symptoms:
Bilateral conjunctival infection, mouth/throat changes, hand and feet changes, rash, swollen glands
How to manage kawasaki disease?
High dose aspirin initially then move onto low dose
IVIg-Gamma globulin (after which symptoms will improve after 36 hours)
If IVIg failure, consider steroids
What should you be aware of when giving children aspirin?
Reyes syndrome: persistent vomiting, lethargy, liver and brain damage
Presentation of measles
Initially coryzal sxs, fever, small grey-white spots in mouth (Koplik spots) lasting a few days
2-4 days later= maculopapular rash on head/neck and spreads to rest of body
Management of measles
Self limiting viral illness and usually clears in about 7-10 days
Mx: stay at home for at least 4 days from when rash first appears, antipyretic, fluids, gently clear crust from eyes, hot steam
PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months
What are some complications of measles?
Common= dehydration, otitis media, conjunctivitis, laryngitis, airway infections, febrile seizures Uncommon= hepatitis, squint, meningitis encephalitis Rare= optic neuritis, heart and nerve problems, subacute sclerosing panencephalitis several years after
What causes chicken pox?
Varicella zoster virus- commonly before age 10
Presentation of chicken pox?
Fever aches and headache for about a day before the rash: spots in crops become small itchy blisters and then scab over
May lose appetite and have feeding problems
Management of chickenpox?
AVOID aspirin (reye's syndrome) Antipyretic, emollient, sedating antihistamine at night, keep nails short, dress comfortably, fluids Stay off school until all blisters crusted over
If an at risk group eg under 1 month, immunocompromised, severe heart/lung disease=aciclovir or vaccination
When are you infectious with chickenpox?
7-21 day incubation
Infectious from 2 days before spots appear until all blisters crusted over
What type of infection is rubella? When is peak incidence?
Viral. Mainly in spring and early summer
Presentation of rubella?
Prodromal phase: low grade fever, headache, mild conjunctivitis, anorexia, rhinorrhoea
Rash occurs 14-17 days after exposure: pink discrete macules that coalesce behind ears and on face, spreads to trunk then extremities
Lymphadenopathy-cervical, suboccipital, post auricular
Management of rubella?
PCR is gold standard diagnosis
Keep out of school for 4 days after rash appears, antipyretics, ask re contact with pregnant women
PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months
What causes diphtheria?
Gram positive aerobic bacillus Corynebacterium diphtheriae causing acute upper resp tract infection
Presentation of diphtheria?
Early sxs=coryzal
Nasal discharge- watery then purulent and bloody
Membranous pharyngitis with fever, enlarged cervical LNs, oedema of soft tissues= bull neck appearance
Cutaneous infection: pustules rupture causing punched out ulcers often on lower legs, feet and hands, with surrounding oedematous pink/purple skin. Takes 2-3 months to heal leaving a depressed scar
May cause difficulty swallowing, paralysis and HF
Management of diphtheria?
PREVENTION: 6-in-1 vaccination at 2, 3 and 4 months
Antibiotics (eyrtho/azithro/clarithro/penicillin), reinforcing vaccine dose, manage contacts with abx prophylaxis
Booster vaccinations: first between 3.5 and 5 years, second between 13-18
Pathophysiology of scalded skin syndrome?
Staphylococcal disease. Also known as Ritter disease and Lyell disease
Affects under 5s, especially neonates normally
Exotoxins from S. aureus bind to desmosomes, causing skin cells to become unstuck- causes red blistering skin
