Paediatrics Flashcards
Paediatrics core conditions questions
Definition of chronic constipation
2 or more of the following in last 8 weeks:
- Less than 3 bowel movements a week
- 1 episode of incontinence a week
- Stools blocking toilet
- Stool palpable in abdomen
- Retentive posturing/ with-holding behaviours
- Painful defecation
What percentage of children are affected by constipation?
5-30%
What percentage of constipation is idiopathic?
90%
Red flags for constipation- action taken
Don’t treat constipation, refer
Red flags for constipation
- Failure to pass meconium within 48h of birth
- ‘Ribbon stools’ suggests anal stenosis
- Failure to thrive
- Gross abdominal distension
- Lower limb neurology
- Urinary incontinence
- Signs of spina bifida (sacral dimple, naevi, hairy patch)
- Abnormal anorectal anatomy
- Perianal bruising/ fissures (?sexual abuse)
- Perianal fistulae/ abscesses
Amber flags for constipation: action performed
Treat constipation and initiate further relevant Ix
Amber flags for constipation
Faltering growth
? Maltreatment
Peri-anal streptococcal infection
When should a referral be made for constipation not responding to treatment
4 weeks (under 1s) 3 months (older children)
First line treatment for constipation
Polyethylene glycol 3350 + Electrolytes (Movicol Paediatric Plain)
Dose escalation over 2 weeks if impaction
Second-line add-ins for constipation
Stimulant laxatives e.g. Sodium picosulfate, Bisacodyl, Senna, Docusate
Which medications should only be added for impaction when everything else has failed?
Rectal medications/ enemas e.g. Sodium citrate
Signs suggesting hypernatraemic dehydration in Gastroenteritis
Jittery movements Increased muscle tone Hyperreflexia Convulsions Drowsiness or coma
When may stool MC+S be indicated in gastroenteritis?
Sepsis Blood or mucous in stools Immunocompromised Caught abroad Not improving within 7 days
When are antibiotics indicated for gastroenteritis?
Septicaemia Salmonella ( under 6 months) C. difficile giardiasis dysenteric shigellosis dysenteric amoebiasis cholera
Dose of Oral Rehydration Solutions
50ml/kg over 4 hours + maintenance
How long does gastroenteritis last?
diarrhoea usually lasts for 5–7 days, and in most it stops within 2 weeks
vomiting usually lasts for 1–2 days, and in most it stops within 3 days
Important investigations in gastroenteritis
Glucose (children at much higher risk of hypoglycaemia)
Risk factors for Gastro-oesophageal reflux
Cerebral palsy Neurodevelopmental disorders Obesity Family history Congenital atresia Pyloric stenosis
Why are infants at a high-risk of reflux?
- Short, narrow oesophagus,
- Delayed gastric emptying
- Immature lower oesophageal sphincter that is slightly above rather than below the diaphragm
- Liquid diet
Indications for investigation in Gastro-oesophageal reflux?
Unexplained feeding difficulties Distressed behaviours FTT Chronic cough Hoarseness Pneumonia
Complications of GOR
Oesophagitis Recurrent aspiration/ pneumonia Sandifer Syndrome (Dystonic neck posturing) Frequent OM Dental erosion
What is posseting
Milk coming out of the babies mouth after feeding (only a small amount)
Thickeners for GOR in bottle fed babies
rice starch, corn-starch, locust bean gum or carob bean gum
Indications for 4 week trial of a PPI/ H2 antagonist in GOR
- unexplained feeding difficulties (refusing feeds, gagging or choking)
- distressed behaviour
- faltering growth
A common complication of viral gastroenteritis
Transient lactose intolerance
Extra-abdominal causes of acute abdominal pain in children
Lower lobe pneumonia
URTI
Testicular torsion
Hip and spine
Meckel’s diverticulum as a cause of acute abdominal pain- rule of 2’s
Is the most common congenital abnormality of the GI tract. Presentation includes PR bleeding, obstruction, volvulus, intussusception, inflammation, perforation
2% of population affected
2% of these are symptomatic
Lesions are 2cm long
Normally 2 feet from the Ileocaecal valve
2/3 have ectopic tissue, of which there are two types (gastric and pancreatic)
In what percentage of children is a structural cause of recurrent abdominal pain identified?
10%
Symptoms that suggest organic disease in recurrent abdominal pain
Epigastric pain at night (peptic ulceration) Jaundice Haematemesis Diarrhoea Weight loss FTT Vomiting Dysuria/ secondary eneuresis Billous vomiting Abdominal distension
Abdominal migraine
Abdominal pain associated with headaches
Midline pain, vomiting, facial pallor
Common in families with FH of migraine
IBS in children
Common when family history or psychosocial issues
- Abdominal bloating relieved by defecation
- Explosive stools
- Feeling of incomplete empyting
- Constipation alternating with normal stools
Risk factors for Coeliac disease in children
T1DM
Thyroid disease
Turner’s syndrome
Family history
Protective factors against coeliac disease in children
Breastfeeding alongside gluten introduction
Investigations for Coeliac disease
Antibody testing whilst eating gluten:
- Tissue Transglutaminase (tTGA) antibodies
- IgA Endomysial antibodies (EMA)
Less reliable in children under 18 months
Other tests:
Endoscopy (villous atrophy)
Ferritin, B12, Hb blood tests
Presentation of colic
Paroxysmal crying with legs pulled up, occurring 3+ hours for 3+ days of the week
Suggests:
- Feeding difficulties
- Inadequate milk supply
- Hungry baby
- Relationships/ bonding issues
Management of colic
Advice and reassurance on:
- Stress reduction with feeding
- Low allergen diet
- Allow baby to finish first breast first before beginning second breast
Presentation of a strangulated hernia
Vomiting
Irritability
Tachycardia
Oedematous/erythematous skin
Management of umbilical hernias
96% will close by 3 months if less than 0.5cm
If still present aged 3-5, unlikely to close so will be repaired surgically for cosmetic reasons
9X more common in black than caucasian children
Presentation of intussusception
Paroxysmal SEVERE colicky pain and pallor (particularly around mouth).
Drawing up of the legs
Early profuse vomiting which becomes bile-stained quickly
Refusal of feeds
Dehydration ± shock and pyrexia
Mucoid and ‘redcurrant jelly’ bloody stools (later sign)
Signs of Intussusception
Target sign on USS
Dance’s Sign (absence of bowel in right lower quadrant)
Why is jaundice rare in older infants
Most causes are detected upon the Guthrie test
Liver causes of jaundice in children
- CF
- Wilson’s disease
- Post-viral hepatitis
- IBD
- Primary sclerosing cholangitis
- A1 antitrypsin deficiency
- Bile duct lesions
Causes of conjugated jaundice in children
- Biliary atresia
- Urinary Tract Infection
- Hypothyroidism
- Neonatal Hepatitis Syndrome
Mesenteric adenitis presentation and management
Abdominal pain (commonly central or in RIF) following a viral infection
- Fever, malaise, nausea and diarrhoea all common
- Preceded by viral or bacterial infection ? coryzal symptoms
Treat with PRN analgesia but may require surgical opinion
Risk factors for Pyloric stenosis development
Family history Primips Boys Erythromycin exposure Prematurity
ABG findings in pyloric stenosis with dehydration
Metabolic alkalosis with hypochloraemia and hypokalaemia
USS findings in pyloric stenosis
Muscle thickness >4mm
Muscle length > 18mm
Absence of fluid passage past the sphincter, despite gastric peristalsis
Which side is more commonly affected in testicular torsion
Left
Why might hormonal injections be used in undescended testes?
HCG injections can detect presence of impalpable testes as it induces a rise in serum testosterone
Hepatitis A in children
Follows close contact or travel to an endemic area
- Asymptomatic or mild self-limiting illness with full recovery in 2-4 weeks
- Symptomatic treatment ± - Human Normal Immunoglobulin (HNIG) prophylaxis for close contacts
Hepatitis B and C in children
May be acquired in travel to areas with high-prevalence e.g. Sub-Saharan africa, South america, Far east
- May be acquired from maternal infection, blood products
Hep B is normally self resolving in older children. In younger children it may symptomatic but risk of chronic disease is 90%
Hep C is slower and more chronic however 75% will inherit chronic disease
- Pharmacological management reduces the risk of cirrhosis and hepatocellular carcinoma and liver failure.
- Pegylated Interferon alpha-2a (blocks viral protein synthesis) or antivirals (Lamivudine)
Autoimmune Hepatitis
May present as an acute or chronic hepatitis, or in a similar way to cirrhosis. Corticosteroid management is needed. Some children may need a liver transplant.
Fulminant hepatitis
Associated most commonly with paracetamol overdose causing necrosis and loss of liver function. LFTs and clotting are deranged, cerebral oedema occurs. Treat by managing blood glucose, haemorrhage (Vit K, FFP, Cryoprecipitate), and preventing sepsis
Medical conditions associated with diabetes in children
Family History- PATERNAL > maternal HLA-DR3/4 genes Hypothyroidism Addison’s Disease Coeliac Disease Rheumatoid Arthritis Cystic Fibrosis
Maturity-Onset Diabetes of the Young (MODY)
Represents 5% of all diabetes in white children
Is an autosomal-dominant presentation
MODY3 most common subtype and is similar to T2DM
Blood glucose diagnosis of Type 1 diabetes
Random >11 mmol/L
Fasting > 7 mmol/L
Blood glucose control targets in T1DM
4-7 mmol/L before meals/ waking
5-9 mmol/L after meals
HbA1c 48mmol/mol (6.5%)
Basis of insulin calculations for T1DM
- 1U reduces blood glucose by 5mmol/L when over 7mmol
- 1U accounts for 10g of carbohydrates consumed
Important complications of DKA in children
Cerebral oedema Leucocytosis Infection Creatinine Hyponatraemia Ketonuria Hypokalaemia VTE
Growth adjustment considerations to make in premature babies
Should reach normality in:
HC at 18 months
Weight at 24 months
Height at 40 months
Weight change in first week of life
Babies may lose up to 10% weight in first week of life
Normally re-gained by 3 weeks
NICE recommendations for identifying faltering growth/ FTT:
- Fall across 1+ weight centiles if birth weight below 9th centile
- Fall across 2+ weight centiles if birth weight between 9th and 91st centiles
- Fall across 3+ weight centiles if birth weight above 91st centile
- Current weight below 2nd centile regardless of birth weight
When should children be admitted for FTT?
If under 6 months and severe FTT
Most common enzyme deficiency in Congenital Adrenal Hyperplasia
21 Hydroxylase (needed for cortisol synthesis; leads to elevated ACTH and testosterone production)
Metabolic disturbances in Congenital Adrenal Hyperplasia
Raised 17⍺ Hydroxyprogesterone Low Na+ High K+ Metabolic Acidosis Hypoglycaemia
Acute Adrenocortical Crisis
Vomiting, dehydration, abdominal pain, lethargy
Treat with hydrocortisone, saline, glucose, fludrocortisone
Presentation and Management of Congenital Adrenal Hyperplasia
Female genital virilisation
Penis enlargement and precocious puberty in boys
Tall stature (non-salt losers)
muscular build, adult body odour, pubic hair
Requires lifelong glucocorticoid treatment to suppress ACTH and surgical correction of ambiguous genitalia
Which gene promotes formation of male genitalia (and if not present, female genitalia are formed)?
SRY gene
Production of Anti-Mullerian hormone occurs
Causes of Disorders of Sexual Development/ Ambiguous genitalia in genetic females
CAH- virilisation
May have:
Clitoral hypertrophy of any degree
Vulva with single opening
Causes of Disorders of Sexual Development/ Ambiguous genitalia in genetic males
Androgen insufficiency
Gonadotrophin insufficiency e.g. Prader-Willi, Congenital Hypopituitarism, Ovotesticular disorder
May have:
Severe hypospadias with bifid scrotum
Undescended testes with hypospadias
Bilaterally non-palpable testes in a full-term male infant
Definition of precocious puberty
Puberty beginning before:
- 8 in girls
- 9 in boys
Path of puberty in males
Testicular enlargement first sign
Growth spurt 18 months later
Pubic hair development
Puberty in females
Breast development first sign
Menarche follows ~2.5 years after breast development (at stage 4 most commonly)
Pubic hair development
Causes of Gonadotrophin-Dependent precocious puberty
90% idiopathic due to premature activation of Hypothalamic - Pituitary axis
Rare causes: CNS tumours/ pathology
Causes of Gonadotrophin-Independent precocious puberty
Production of sex hormones from excess steroids Familial or Idiopathic (usual cause in females) Gonadal tumour in males Stress CNS tumours Craniopharyngioma Thyroid disorders Meningoencephalitis 21-Hydroxylase Deficiency (CAH) McCune Albright Syndrome
Delayed puberty definition
Absence of puberty features by:
- 14 in girls
15 in boys
What is Tanner Stage 1 of puberty?
No puberty occurred
Low gonadotrophin causes of Delayed puberty (hypogonadotropic hypogonadism)
o CF o Severe asthma o Crohn’s Disease o Organ failure o Anorexia nervosa o Starvation o High exercise levels o Panhypopituitarism o IC tumours o Kallmann Syndrome
High gonadotrophin causes of (hypergonadotropic hypogonadism)?
Klinefelter’s Syndrome
Turner Syndrome
Gonadal damage
Foetal thyroid function
Reverse T3 production (largely inactive)
Surge in TSH leads to high T3 and T4 at birth
Features of acquired hypothyroidism specific to children
Growth Failure
Delayed puberty
Slipped upper femoral epiphysis
Learning difficulties
Conditions associated with acquired hypothyroidism
Down’s and Turner’s Syndromes
Features of Congenital Hypothyroidism specific to children
Failure to thrive and delayed development Feeding problems Constipation Reduced crying Prolonged jaundice Coarse facies Umbilical hernia Cold, mottled dry skin Narrow palpebral fissures Depressed nose bridge Swollen eyelids Large fontanelles
Biochemical defect in Phenylketonuria (PKU)
High phenylalanine due to deficiency of Phenylalanine Hydroxylase enzyme or its Biopterin co-factor
Features of phenylketonuria
Fair hair, eczema, musty odour, developmental delay, mental impairment, fits, eczema, microcephaly
Foods to avoid/ restrict in phenylketonuria
Meat, dairy, rice, pasta, aspartame
Definition of short stature
Height 2SD below age-adjusted mean- often below 2nd or 3rd centile
Most common cause of short stature
Constitutional (80%)
Indications for Somatropin (Synthetic Growth hormone in Short stature
Somatropin increases growth VELOCITY to 50% of baseline within first year of treatment
o Have GH deficiency
o Have Turner’s
o Have Prader-Willi
o Have CKD
o SFGA with subsequent growth failure at 4 or older
o Short stature homeobox-containing gene deficiency
NICE definitions of obesity
- BMI >91st centile – Overweight
- > 98th centile – Obese
- > 99.6th centile – severely obese
Consequences of obesity in children
Slipped Upper Femoral Epiphyses
Blount’s disease (a development abnormality of the tibia resulting in bowing of the legs)
Musculoskeletal pains
Poor self-esteem, bullying
sleep apnoea
benign intracranial hypertension
increased incidence of type 2 diabetes mellitus, hypertension and ischaemic heart disease
etc.
Indications for drug treatment of obesity in children (Orlistat)
Over 12, significant physiological or psychological co-morbidities
Age which febrile seizures occur
6 months to 6 years
Features of a Simple febrile seizure
Isolated tonic-clonic rhythmic seizure activity Lasts LESS than 15 minutes Complete recovery within 1 hour No recurrence in 24 hours No recurrence in the same illness
Features of a Complex febrile seizure
Any of:
Lasts longer than 15 minutes Focal seizure activity Incomplete recovery within 1 hour Recurrence within 24 hours Recurrence within the same illness
Symptoms seen in a simple febrile seizure
Lasts 2 -3 minutes Rolling back of the eyes Foaming at the mouth SOB, pallor, cyanosis Post-ictal drowsiness lasting less than 1 hour
Fever- temperature in febrile seizures
38 degrees
Reasons to consider hospital admission after febrile seizure
First febrile seizure Seizure lasting over 15 mins Focal seizure Seizure recurring within same febrile illness within 24 hours Incomplete recovery after one hour Child under 18 months
Antenatal risk factors for cerebral palsy
Chorioamnionitis
Maternal respiratory/ urinary infection
TORCH infection
Preterm birth (risk of periventricular leukomalacia)
Perinatal risk factors for cerebral palsy
Placental abruption Hypoxic-Ischaemic birth injury Neonatal encephalopathy Neonatal sepsis Low birth weight Low APGAR score at birth
Postnatal risk factors for cerebral palsy
Meningitis/ Encephalitis Head Trauma Hypoglycaemia Hydrocephalus Hyperbilirubinaemia
General features of Spastic Cerebral palsy
Most common types UMN lesions (pyramidal, corticospinal) Brisk tendon reflexes and extensor plantar responses Velocity-dependent tone
Hemiplegic spastic cerebral palsy
Affects either hand or leg (monoplegia), or both unilaterally
- Tiptoes walking
- Circumduction gait
- Growth arrest in extremities
- Pronated forearm and fisting
Diplegic spastic cerebral palsy
Commonly caused by periventricular leukomalacia
All four limbs affected but to a lesser extent than with quadriplegia
- Arm deficits are greater with functional use
- Scissoring gait
Quadriplegic spastic cerebral palsy
The most severe spastic CP
- Microcephaly with poor head control
- Seizures
- Opisothonus
- Moderate to severe intellectual impairment
Ataxic cerebral palsy
Early trunk and limb hypotonia
Impaired force, rhythm and accuracy of movements
Delayed motor and intellectual development
Behavioural and communication problems
Bladder and bowel dysfunction
Dyskinetic cerebral palsy
Features abnormal recurring movement disorders Commonly caused by HIE and Kernicterus - Floppiness - Poor trunk control - Delayed motor development
3 patterns: Athetosis, Dystonia, Chorea
Chorea-pattern cerebral palsy
- Irregular sudden non-repetitive movements
- Reduced tone
Dystonic-pattern cerebral palsy
Involuntary and sustained muscle contractions
Athetosis- pattern cerebral palsy
Slow ‘writhing’ distal movements e.g. finger fanning
Childhood Absence Epilepsy
Common between 5 and 12 years of age
May be triggered by hyperventilation
Automatisms common e.g. lip smacking, eyelid flickering, staring
Episodes lasting 5-20 seconds
Usually remit in adolescence without treatment
Juvenile Myoclonic Epilepsy
Most common in teenage girls
Common early morning
Tonic-clonic seizures, absence seizures
40% are photosensitive, also triggered by sleep deprivation and alcohol
Rx: Usually requires lifelong antiepileptics
Infantile spasms and West Syndrome
Infantile Spasms:
Common at around 5 months of age
Head nodding and arm-jerk attacks
Occur every 3-30 seconds
West Syndrome:
As above, plus motor regression and typical EEG pattern
Rx: Prednisolone 1st line, Vigabatrin 2nd line
Childhood Epilepsy with Centro-Temporal Spikes (CECTS)/ BECTS/ Benign Rolandic Seizures
Peak at 9-13 years
Are nocturnal
Typically last less than 5 minutes
Hemifacial paralysis and paraesthesia, salivation/ drooling
Medications available for migraines
Simple analgesia
Anti emetics in over 12s (Domperidone, Prochlorperazien)
Triptans in over 6
Prophylactic Propanolol, Topiramate, Pizotifen
Pharmacological treatment options for Tics (and short-term for Tourette’s)
Risperidone, Clonidine
Inheritance pattern of Duchenne Muscular Dystrophy
X Linked Recessive
Deficiency of Dystrophin protein
When is daytime continence usually achieved
24-48 months
When is night continence achieved
3-4 years
Over what age may Enuresis alarms be considered
7 years
Continue use until 2 dry weeks
Asymptomatic bacteruria
10^5 Colony Forming Units/mL without any symptoms of UTI
What is a MCUG- when used
Micturating Cysturethrogram
Used in under 1s to diagnose Vesico-ureteric reflux in recurrent UTI. The child is catheterised and given prophylactic antibiotics; scanned with contrast whilst urinating
Commonly only performed in under 1’s
What is a DMSA scan
Di-mercaptousuccinic acid Scintigraphy Scan
Normally performed 6 months after acute infection, it detects renal scarring damage by injection of radioisotope and then scanning.
What is the most common cause of AKI in children
Haemolytic Uraemic Syndrome
Most common causes of HUS
Gastroenteritis- E coli 0157 or Shigella
Triad of features in HUS
AKI
Thrombocytopaenia
Microangiopathic Haemolytic Anaemia (Coomb’s Negative)
What is an early sign of HUS
High blood LDH
Key signs of HUS
Profuse, Bloody Diarrhoea
Followed by Fever, Abdo pain, vomiting, Oliguria
Triad of features in Nephrotic Syndrome
Proteinuria
Oedema
Hyperlipidaemia
Most common cause of Nephrotic Syndrome
Minimal change nephropathy
Symptoms of Nephrotic syndrome
Peri-orbital oedema Limb/ scrotal/ vulval oedema Ascites SOB Cloudy urine with protein casts
Indications for renal biopsy in children with haematuria
Normal renal function
Significant persistent proteinuria
Recurrent macroscopic haematuria
Abnormal complement levels
Definition of haematuria
10 RBCs per high power field
Diagnostic criteria for eczema
Itching plus 3 from:
- Age appropriate distribution
- Personal hx/ 1st degree FH if under 4
- Dry skin in last 12 months
- Asthma or allergic rhinitis
- Onset under 2
Treatment of impetigo
Fusidic acid/ Mupirocin
Oral e.g. flucloxacillin if severe/ Bullous
What condition is Gower’s Sign associated with
Duchenne Muscular Dystrophy
Patient has to use arms to ‘walk up’ their body- its a sign of proximal muscle weakness
Common precipitants of Stevens-Johnson Syndrome
Allopurinol Anticonvulsants Sulphonamides NSAIDs Penicillins Imidazole Antifungals Sertraline
Most common organisms of septic arthritis in neonates
Group B Streptococci
Streptococcus pyogenes
Most common organism of septic arthritis in older children
Staphylococcus aureus
Haemophilus influenzae
Antibiotics for Septic Arthritis: empirical prescribing
Flucloxacillin 2 weeks IV
Clindamycin if penicillin allergic
Vancomycin: MRSA involved
Ceftriaxone/ Cefotaxime: Gonococcal or gram negative infection
Kocher Criteria for septic arthritis
Non- Weight Bearing
ESR >40
Temperature 38.5+
WCC 12000 cells/mm3 +
1 present: 3% risk
2 present: 40% risk
3 present: 93% risk
4 present: 99% risk
Barlow Manoeuvre
Detects an unstable hip by DISLOCATING it (Barlow Breaks)
Hip dislocated inferiorly with the thumb and usually clicks if positive
Ortolani Manouevre
Relocates a subluxed or partially dislocated hip
Test is negative if dislocation is irreducible
Which babies require an ultrasound at 6-8 weeks for DDH
Those which are still breech by 36 weeks First degree relative with hip problems as a child Breech presentation at 36 weeks Twins Prem Hip feels unstable
Management of DDH
Bracing with a Pavlik Harness for children under 6 months
Surgery/ open reduction thereafter, or if the bracing fails
Joints most commonly affected by septic arthritis in children
Knee > Hip > Ankle
Common precipitating infections for Transient Synovitis/ Irritable hip
Viral URTIs
Gastroenteritis
Osteochondritis dissecans
Cracks in the cartilage due to AVN
- Pain and swelling of joint
- Locking during movement
Chondromalacia patellae
Overuse cartilage injury
- Runner’s Knee
Osgood- Schlatter Disease
Inflammation of the patellar ligament at the tibial tuberosity
- Painful bump below the knee, worse with activity (running, jumping)
- Most commonly males 10-15
Red flags of a limping child
- Pain waking at night (malignancy)
- Redness, swelling or stiffness of the joint or limb (infection or inflammatory joint disease)
- Weight loss, anorexia, fever, night sweats or fatigue (malignancy, infection or inflammation)
- Unexplained rash or bruising
- Limp and stiffness worse in the morning
- Severe pain, anxiety and agitation after a traumatic injury (compartment syndrome)
Management of Perthe’s Disease
Conservative if Under 8 or bone age <6
Otherwise: surgery- osteotomy
Physio and strengthening
Reactive arthritis
Transient joint swelling following an enteric infection e.g. Salmonella, campylobacter etc.
- Low fever plus joint swelling
- NSAIDs
XR findings in Slipped Upper Femoral Epiphysis (SUFE)
Widening of the growth plate (posteriorly)
XR findings in Perthe’s Disease
Reduction in epiphysis size and lucency
Fragmentation and destruction
Joint space widening
Loss of shenton’s lines
What does a high ANA predict in JIA
Uveitis risk
Features of Oligoarticular JIA
Most common (50%)
1-4 joints affected (called extended if more are subsequently affected)
Knee and ankle most common
Normally girls, under 6
Features of Polyarticular JIA
5+ joints affected
It may be RF positive or negative
Features of Systemic JIA
Arthritis with 2+ weeks of daily fever Other Sx: - Rash - Lymphadenopathy - Hepatosplenomegaly - Serositis - Salmon pink rash
Features of Psoriatic JIA
Arthritis then Psoriasis
Dactylitis, onycholysis
Normally asymmetrical
Features of Enthesitis Related JIA
Inflammation at tendon/ ligament sites
Features of Iron Deficiency
anaemia and fatigue, impaired cognitive development and reduced growth
Features of Zinc Deficiency
Immune deficiency, acrodermatitis, increased childhood illness and death
Features of Iodine Deficiency
Causes Goitre, hypothyroidism, growth restriction
Features of Vitamin A deficiency
Night blindness, immune deficiency, increased childhood illness and death
Bloods findings in Ricketts
Increased Alk Phos and PTH
Low Calcium and Phosphorus
Guthrie Test- what’s included
- Cystic Fibrosis
- Sickle Cell Disease
- Congenital Hypothyroidism
- Phenylketonuria (PKU)
- Medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
- Maple-Syrup Urine Disease
- Isovaleric Acidaemia
- Glutaric Aciduria Type 1
- Homocystinuria (pyridoxine unresponsive) (HCU)
Features of DiGeorge Syndrome (CATCH-22)
C: Cardiac Abnormalities A: Abnormal face T: thymic hypoplasia C: Cleft Palate H: Hypocalcaemia
22: 22q11 microdeletion
What percentage of Down’s Syndrome is due to Meiotic Non-Disjunction
94%
Which chromosome is normally implicated in a T21 Translocation?
Chr 14
Most common cardiac issues in Down’s Syndrome
VSD or Secundum ASD
Tetralogy of Fallot
Isolated PDA
What is a concomitant squint
A non-paralytic squint
- Normally an esotropia
- Refractive error in one or both eyes, commonly due to a neurodevelopmental delay
What is a paralytic squint
- Motor nerve paralysis e.g. due to a SOL
- Squint varies with gaze
What is a manifest squint
- Grossly present on observation
What is a latent squint
A squint that isn’t visible on inspection and is produced by the cover test
Which type of squint are more severe
Tropias are more severe than phorias
Medical conditions associated with Autism development
Fragile X
Neurofibromatosis Type 1
Tuberous sclerosis
Phenylketonuria
What age must autism be diagnosed before
36 months
Three domains of Autism
Social Interaction Impairment
Language and Communication Skills
Rigidity of Thinking
Mainstay of autism treatment
Applied Behavioural Analysis (ABA)
- Stops ritualistic behaviours
- Improves sleep and social contact
- Requires 25-30hrs a week to be successful
What is Retinopathy of Prematurity associated with
High flow O2 use in premature babies
Treated with laser therapy
Which conditions are associated with cataracts in children
Trisomy 21
Turner’s Syndrome
Neurofibromatosis Type 2
Juvenile cataracts
Screening for developmental delay
- Birth Neonatal Examination: Eyes (red reflex), heart, hips, automated screening of hearing
- Guthrie Screen at 1 Week (PKU, Hypothyroidism, CF, Hb-opathies, Acylcarnitine)
- 8 week general GP examination
- 4-5 years (orthoptist)
- School Entry: Height and weight, hearing
Which medication may be offered short term for Conduct Disorder or ODD in children?
Risperidone
Monitoring and side effects of Methylphenidate
Initial BP, height and weight, ECG
SEs: Growth restriction (1%), loss of appetite
When is attachment behaviour maximal
18-36 months
Average birth weight of a child
3.3kg
Average head circumference (HC) of a child at birth
35 cm
VACTERL Conditions
Vertebral Anorectal Cardiac Tracheo-Oesophageal Renal Limb
Symptoms of anxiety in children
Dizziness Fainting Rapid Breathing Butterflies Nausea Sweating Palpitations and Tachycardia Recurrence of thought
T waves on paediatric ECG
Upright for first week of life
Inverted until adolescence
Axis of paediatric ECG
May be Right axis deviation
QRS complex in paediatric ECG
Dominant R waves V1-V3
Shorter QRS
Dominant RV means that if V6 overlaps V5, there is a chance of LVH
Features of an Innocent Murmur
- Asymptomatic patient
- Soft blowing murmur
- Systolic murmur only
- Sternal edge (left)
- Sensitive to position e.g. standing up
- Small (doesn’t radiate)
- Single (no associated clicks or gallops)
What is Eisenmenger’s Syndrome
When the pressure of the Pulmonary artery exceeds the LVH, blood begins to shunt back the other way. Usually appears 9 months after development, and the only treatment is a complete heart-lung transplant.
Murmur associated with ASD
Systolic murmur and fixed split S2
At upper left sternal edge
Murmur associated with VSD
Systolic murmur
At lower left sternal edge
Murmur associated with PDA
Machinery like murmur
Murmur associated with coarctation of the aorta
Ejection systolic murmur
Murmur associated with TGA
No murmur, but loud S2
Which congenital heart abnormality is associated with Turner’s Syndrome
Coarctation of the aorta
Which genetic conditions are associated with tetralogy of fallot?
Digeorge Syndrome
Foetal alcohol syndrome
Phenylketonuria
22q11 microdeletion syndrome
Common causes of neonatal jaundice in first 24 hours
Infection (TORCH, sepsis)
ABO incompatibility (developed countries)
Rhesus incompatibility (developing countries)
Hypothyroidism
Hereditary spherocytosis
G6PD deficiency
Persistent neonatal jaundice after 2 weeks- most common cause
Biliary atresia
Physiological jaundice causes
Cephalohaematoma
Prematurity
Breastfeeding jaundice/ breastmilk jaundice
Kernicterus symptoms
High bilirubin levels (360+) where bilirubin crosses the BBB
Lethargy, poor feeding, hypertonicity, opisthonus
Investigations of pathological jaundice
Transcutaneous Bilirubin first
Serum bilirubin if:
- 250+ on transcutaneous BR
- Under 24 hours old
- Gestational age under 35 weeks
Indications for IVIG in jaundice
ABO or Rh incompatibility
Rapidly rising serum BR
Birth asphyxia typical criteria
Cord Blood pH <7.05
APGAR 0-5 at 10 minutes
Hypoxic ischaemic encephalopathy
Treament for infantile haemangioma
Propanolol (reduces blood flow to the lesion)
Treatments for Birth Asphyxia/ HIE
Monitor/ manage seizures with CFAM
Therapeutic hypothermia
Fluids (careful of renal impairment)
ABO blood group incompatibility
Mother usually O, baby A
Can’t be detected antenatally
Do Direct coomb’s test
Bronchopulmonary Dysplasia (BPD)
Complication of Respiratory Distress Syndrome
Due to pressure/ volume trauma from oxygen therapy
Defined as needing oxygen support past 36 weeks gestational age
TORCH infection
Toxoplasmosis Other- Syphilis, VZV, Parovirus B19 Rubella Cytomegalovirus Herpes
Talipes Equinovarus features
Inversion
Adduction of the forefoot
Inability to evert/ dorsiflex
Equinus deformity
Live Vaccines
- Measles (part of MMR)
- Rotavirus
- Polio (IPV)
- Tuberculosis (BCG)
- (Yellow Fever)
Infective organisms of sepsis in children
Neonates: Group B streptococcus, Listeria, E. coli
All: Meningococcus, Pneumococcus
Amber (intermediate) criteria for assessing illness in children
Decreased activity and not responding to normal cues
Nasal flaring
Tachypnoea, increased RR, crackles, reduced O2 sats
Tachycardia
Cap refill 3+ seconds
Reduced urine output
Poor feeding
Dry mucous membranes
Fever, rigors
Limb or joint swelling, on non-weight bearing
Temp 39+ in children 3-6 months
Red criteria for assessing illness in children
Pale/ mottled/ ashen/ blue Not responding to cues Grunting Tachypnoea 60+, chest indrawing Reduced skin turgor Seizures Focal neurology Status epilepticus Neck stiffness Bulging fontanelles Temp 38+ in children under 3 months
Key indications for an LP in septic children
Infants under 1 month
Infants 1-3 months who appear unwell, or with a WCC <5 or 15+
Petechia/ purpura definitions
Petechiae: less than 2mm
Purpura: 2-10 mm
Echymosis: 10mm+
Idiopathic Thrombocytopenic Purpura: key features
2-10 year olds, follows 1-2 weeks after a viral infection
Easy bruising, and a petechial/ purpuric rash over bony prominences
Gum/ nose bleeding
Low platelets (<40 x 10^9/L)
Normally self-resolves but treat if low platelets/ chronic (20%).
- Oral steroids ± IV Ig and venous anti-D
Henoch Schonlein Purpura: key features
4-6 year olds, follows after infection (mycoplasma, EBV, Strep)
Purpuric rash distributed to buttocks, legs and ankles; becomes raised within 24 hours Arthritis and arthralgia Abdominal pain ± GI bleeding Scrotal pain Glomerulonephritis
Diagnosis normally clinical but may need to r/o sepsis
Predominantly supportive treatment (NSAIDs and fluids), consider steroids if severe
- Children should ideally get follow up 6-12 months later to detect renal complications (25%)
Chickenpox- viral features
Incubation 10-21 days
Infectious 1-2 days before rash
Infectious until lesions crusted over (exclude from school until then)
Most common cause of Ophthalmia neonatorum; treatment
Chlamydia
Treat with saline bathing
Features of IgE mediated food allergies
Acute with rapid onset
Urticaria, angioedema, colic, rhinorrhoea, cough, bronchospasm
Features of Non-IgE mediated food allergies e.g. Cow’s Milk Protein Allergy
Atopic eczema Reflux Colic Loose mucosal/ bloody stools Constipation
Investigations for Infectious mononucleosis
Over 12’s:
FBC- differential WCC shows 20%+ atypical lymphocytes
Monospot test
EBV serology
Criteria for Kawasaki Disease
Fever (39+, for 5 days, plus):
- Bilateral non-exudative conjunctivitis
- Cervical lymphadenopathy
- Mucous Membrane changes (cracked lips, strawberry tongue, mouth/ pharyngeal inflammation)
- Polymorphous rash
- Extremity changes (arthralgia, palmar erythema, swelling)
Investigations and Management of Kawasaki Disease
Echocardiogram (detect Coronary A. aneurysm)
Bloods (raised platelets, bilirubin, AST)
Urinalysis- sterile pyuria/ proteinuria
High-dose aspirin for 6 weeks (then antiplatelet dose)
IVIG within 10 days if possible
Measles: clinical features
Cough, coryza and conjunctivitis; plus Koplik’s spots
Incubation period of 10 days
Maculopapular rash starts behind ears and spreads over the body- becoming blotchy and confluent
No school 4 days from rash onset
Complications of measles
Subacute sclerosing panecephalitis (SSPE) Encephalitis Pneumonia Otitis Media Diarrhoea Myocarditis
Mumps features
infective 7 days before and 9 days after parotid swelling starts
incubation period = 14-21 days
Fever, malaise and muscular pain
Parotid swelling
Orchitis
Supportive management but notifiable disease
No school 5 days from swollen glands
Congenital rubella infection features
Sensorineural deafness Congenital heart disease Cataracts Growth retardation Hepatosplenomegaly Cerebral palsy Microphthalmia
Rubella: clinical features
Incubation 14-21 days
Infectious 5 before to 5 after
Fever with maculopapular rash on face spreading to whole body; lymphadenopathy
No school 4 days from rash onset
3 main categories of causes of Iron deficiency anaemia
Inadequate intake (diet, cow’s milk, low Vitamin C)
Malabsorption
Blood loss
Values for Iron deficiency anaemia, and when does it become symptomatic
Neonate – Hb <140g/l
1-12 months – Hb <100g/l
1-12 years – Hb <110g/l
Symptomatic below 60-70
Key symptoms of Fe def anaemia
Fatigue
Slow feeding
Poor behaviour and intellectual function
Conjunctival/ tongue/ palmar crease pallor
Sickle Cell Anaemia: typical symptoms and complications
Anaemia ± jaundice
Infection susceptibility
Splenomegaly
Delayed puberty/ growth restriction Cognitive impairment Tonsillar hypertrophy Cardiac enlargement Heart failure Renal dysfunction Gallstones Leg ulcers
Vaso-Occlusive crisis in SCA
May be exacerbated by cold, dehydration, stress, hypoxia
- Dactylitis
- Mesenteric ischaemia
- Stroke
- Acute chest syndrome (respiratory distress)
Fluids, exchange transfusion
Thalassaemia clinical features
Major is most severe, intermediate less so and minor/ trait is normally asymptomatic. Presentation is:
- FTT
- Extramedullar haemopoiesis
- Skull bossing
- Osteopaenia
- Endocrine failure
Treatments: regular transfusions, splenectomy, bone marrow transplant, iron chelators
Haemophilias
X-Linked Recessive
Haemophilia A more common, F8 deficiency
Higher F8/9 : C ratio indicates a better prognosis
Treatments: Recombinant F8 or F9 ± Desmopressin/ ddAVP for Haemophilia A
Von Willebrands: Autosomal dominant, diagnosed in adolescence. Treat as for haemophilia A
Most common childhood brain tumours
Astrocytoma (40%)
Medulloblastoma (20%)- cerebellar usually
MRI first line
Surgical excision ± adjunct chemotherapy
Neuroblastoma
Neural Crest cell tumour
- Abdominal mass
- Appetite and weight loss
- Bone pain
- Hypertension
- Skin mets (Blueberry muffin baby)
Ix: USS then CT and MIBG bone scans, Urinary catecholamine levels
Wilm’s Tumour
Presents before age 5, FH, Edward’s Syndrome, Beckwith- Wiedemann syndrome
- Abdominal mass ± pain
- Anorexia
- Anaemia
- Haematuria
- Hypertension
Requires nephrectomy then chemo.
Most common leukaemia type in children and its differentiating factors
Acute Lymphoblastic Leukaemia (80%)
- Faster onset
- Younger children
- Males affected more (poorer prognosis)
- No Auer rods
- Low platelets and prolonged Prothrombin time
Leukaemia clinical features
Main signs:
- Anaemia (tiredness, pallor, fever)
- Thrombocytopaenia
- Hepatosplenomegaly
- Lymphadenopathy
Others:
- Petechiae, purpura, bruising
- Headaches and vomiting
- Testicular enlargement
- Bone pain
Hodgkin’s Lymphoma
Presence of Reed-Steenberg cells
- EBV, smoking, immunosuppression, HIV
- Adolescents
Painless lymphadenopathy plus:
- B symptoms
- Cough (airway obstruction)
- Alcohol induced pain
High ESR carries a poor prognosis
Non-Hodgkin’s Lymphoma
No Reed-Steenberg cells
- 5x more common
- Younger children
Lymphadenopathy plus:
- Palpable abdominal masses (hepatosplenomegaly)
- SOB
Pancytopaenia
Moderate acute asthma
PEFR 50% + of expected
Saturations under 92%
Able to talk in sentences
Age-related tachycardia and tachypnoea
Severe Acute Asthma
PEFR 33-50% of expected
Saturations under 92%
Unable to talk in full sentences
Higher tachycardia and tachypnoea
Life-threatening Asthma
PEFR <33% of predicted Saturations under 92% Cyanosis Hypotension Exhaustion Silent Chest Tachycardia
When can an asthmatic child be discharge
Using 6 puffs 4-hourly
Go home with a wheeze plan
Most common organism in Bronchiolitis
RSV
Clinical features of Bronchiolitis
Aged 3-6 months peak but can occur under 12 months
Coryzal prodrome 1-3 days
High-pitched cough, tachypnoea, cracles
Fever and poor feeding
Indications for admission/ referral in Bronchiolitis
RR 60+
Clinically dehydrated
Inadequate intake 50-75%
Saturations <92%
Can consider NG feeding, nasal cannula with humidified oxygen
Most common organisms in croup
Parainfluenza virus
- Type 3 most common
- Type 1 most severe
Clinical features of Croup
Coryzal phase of rhinorrhoea, ST, cough and fever
Barking cough and stridor, often worse at night
Aspects of Westley Score for croup
Chest wall retractions Stridor Cyanosis Level of consciousness Air entry
Under 3: optimal
8+: severe, consider ICU
Management of Croup
Dexamethasone/ Budesonide weight-adjusted
Adrenaline 1 in 1000 if severe
Causative organism in Epiglottitis
Haemophilus influenzae type B
Epiglottitis clinical features
Acute onset Soft whispering stridor with a muffled voice High fever Drooling of saliva/ no fluids Scared
XR findings in Epiglottitis
Thumb print sign
Common organisms in Tonsilitis/ pharyngitis
Group A beta-haemolytic streptococci
EBV
Influenzae
Rhinovirus, adenovirus, HSV1, coronavirus
When to offer an antibiotic for Tonsilitis
Centor score 3+
- Phenoxymethylpenicillin (Penicillin V)
- Corticosteroids
Indications for a tonsillectomy in children
3+ episodes of tonsilitis a year for 3 years
5+ episodes of tonsilitis a year for 2 years
7+ episdoes in a year
Recurrent OM with effusion
Obstructive sleep apnoea
Organism for whooping cough
Bordatella pertussis
Clinical features and management of whooping cough
Spasmodic cough (worse at night) with inspiratory whoops
Epistaxis, subconjunctival haemorrhage
Apnoea
Treat with erythromycin in commenced early
Exclude from school for 3 weeks
Vaccinate mothers
Best signs of pneumonia in children
High fever (39+) Tachypnoea
Others: poor feeding, pleurisy, bronchial breathing, prodromal URTI, respiratory distress
Antibiotics for pneumonia in children
Amoxicillin 1st line
Co-Amoxiclav in neonates
Macrolide if mycoplasma
Antibiotic of choice for AOM
Amoxicillin
Key Neonatal features of cystic fibrosis
Meconium ileus (not passed within 48 hours)
FTT
Prolonged jaundice
Distended bowel loops on AXR
Features of CF in childhood
Frequent respiratory infections Bronchiectasis Persistent loose cough with sputum production Clubbing Chest hyperinflation Nasal polyps Rectal prolapse Steatorrhoea
Longer term complications of Cystic Fibrosis
Male infertility Diabetes Chronic liver disease Osteoporosis Vitamin deficiencies Urinary stress incontinence
Sweat test diagnosis of Cystic Fibrosis
60 mmol/L +
- Need to be 3-4kg
Other causes of false positives:
- Eczema
- Adrenal insufficiency
- Hypothyroidism
- Malnutrition
What does the Guthrie test detect with regards to CF
Immuno-Reactive Trypsinogen
CT findings in cystic fibrosis
Signet rings (bronchioles are larger than adjacent blood vessels)
Infective organisms in cystic fibrosis
S. aureus H. influenzae Pseudomonas aueruginosa Klebsiella Burkholderia cepacian Non tuberculous mycobacteria (once get to this stage, can't transplant)
Chance of a foreign body passing if below the diaphragm
80%
Presentation of inhaled foreign body in children
Choking SOB Wheeze Stridor Refusal to eat Drooling
