paeds part 2 Flashcards
diagnosis of nephrotic syndrome in children?
proteinuria (> 1 g/m^2 per 24 hours)
hypoalbuminaemia (< 25 g/l)
oedema
also hyperlipidaemia
hypercoagulable state (due to loss of anti-thrombin III)
predisposition to infection (due to loss of immunoglobulins)
causes of nephrotic syndrome in children?
minimal change disease (most common)
focal-segmental glomerulosclerosis
post-streptococcal nephritis
histological hallmark of minimal change disease?
fused podocytes
ix of nephrotic syndrome in children?
urine dipstick
FBC, ESR, C3, C4, U&Es
antistreptolysin O or Anti-DNAse
urine MC&S
tx of nephrotic syndrome in children?
if steroid-sensitive (85-90%)- prednisolone 60mg/m2/day until proteinuria ceases
if steroid resistant- diuretics, salt restrictrion, ACEi, NSAIDs
cyclophosphamide +/- ciclosporin
prognosis of nephrotic syndrome in children?
1/3 resolve, 1/3 infrequent relapses, 1/3 frequent relapses
causes of faltering growth?
1) Inadequate intake- impaired suck-swallow, inadequate availability of food
2) Inadequate retention- GORD, vomiting
3) Malabsorption- coeliac, CF, CMPI
4) Failure to utilise nutrients- syndromes
5) Increased requirements- thyrotoxicosis, CF, malignancy, chronic infection
is UTI more common in boys or girls?
boys until 3 months
after that higher in girls
cause of UTI?
E.coli
after that klebsiella, proteus, pseudomonas
presentation of UTI in infants, younger children and older children?
Infants- poor feeding, vomiting, irritability
Younger children- abdo pain, fever, dysuria
Older children- dysuria, frequency, haematuria
mx of UTI in children?
referral to a paediatrician in <3 months
>3 months with upper UTI- consider for admission. If not, cephalosporin or co-amoxiclav for 7-10 days
>3 months with lower UTI- trimethoprim or nitrofurantoin for 3 days
Ix of atypical UTI?
USS of kidneys and urinary tract
MAG 3 or MCUG (micturating cysturethrogram) to detect obstruction and vesicoureteric reflux- backflow of urine into ureter and kidney
DMSA to look for renal scarring
what is haemolytic uraemic syndrome?
caused by an abnormal destruction of red blood cells.
followed prodrome of bloody diarrhoea e.g. E.coli
clog the filtering system of the kidney leading to->
acute renal failure
thrombocytopenia
microangiopathic haemolytic anaemia
S&S of HUS?
Abdominal pain
decreased urine output
normocytic anaemia
Ix of HUS?
Fragmented blood film
stool culture
FBC
tx of HUS?
Supportive
plasma exchange if severe
what is Henoch-schonlein purpura?
vasculitis involving small vessels of the skin
IgA mediated
follows URTI-strep pyogenes
features of HSP?
Purpuric rash (with localised oedema) over buttocks and extensor surfaces of arms and legs
abdominal pain
polyarthritis
feature of IgA nephropathy- haematuria, renal failure
tx of HSP?
supportive
analgesia for arthralgia
prednisolone if severe
self-limiting
tx of nocturnal enuresis?
enuresis alarm
desmopressin
advise of fluid intake and toileting behaviour
what is cystic fibrosis?
AR disorder causing increased viscosity of secretions
due to a defect in CFTR gene which codes for cAMP sodium chloride channel- F508 defect on chr 7
presentation of CF?
Neonatal period- (20%) meconium ileus, prolonged jaundice
Recurrent chest infections (40%)
Malabsorption (30%)- steatorrhoea, failure to thrive
Other features (10%)- liver disease
late features- short stature, DM, delayed puberty, rectal prolapse, nasal polyps, male infertility, female subfertility
diagnosis of CF?
Guthrie heel prick- 6-9 days of life
Sweat test- high chloride ions
Faecal elastase (low)
gene abnormalities in CFTR gene
mx of CF?
Chest physiotherapy several times a day is essential to clear mucus and reduce the risk of infection and colonisation
Exercise improves respiratory function and reserve, and helps clear sputum
High calorie diet is required for malabsorption, increased respiratory effort, coughing, infections and physiotherapy
CREON tablets to digest fats in patients with pancreatic insufficiency (these replace the missing lipase enzymes)
Prophylactic flucloxacillin tablets to reduce the risk of bacterial infections (particularly staph aureus)
Treat chest infections when they occur
Bronchodilators such as salbutamol inhalers can help treat bronchoconstriction
Nebulised DNase (dornase alfa) is an enzyme that can break down DNA material in respiratory secretions, making secretions less viscous and easier to clear
Nebulised hypertonic saline
Vaccinations including pneumococcal, influenza and varicella
CF patients are prone to infections caused by?
staph aureus
pseudomonas aeruginosa
aspergillus
cause of croup?
parainfluenza virus
6 months- 3 years
features of croup?
stridor
barking cough (worse at night)
fever
coryzal symptoms
mx of croup?
dexamethasone 150mg/kg ONCE then reassess
High flow O2
Nebulised adrenaline
cause of bronchiolitis?
RSV
respiratory syncytial virus
most common cause of LRTI in <1 years old
features of bronchiolitis?
coryzal symptoms dry cough increasing breathlessness wheezing, fine inspiratory crackles feeding difficulties
Ix of bronchiolitis?
nasopharyngeal aspiration- immunofluorescence may show RSV
mx of bronchiolitis
supportive- humidified O2, NG feeds, fluids
Palivizumab- monoclonal Ab, IM once a month through autumn and winder for immunocompromised, CHD, CF, down’s
Ix for a child failing to thrive?
Bloods- FBC, U&E, LFT, TFT, Ig, IgA TTG
Urine- MC&S
Stool- MC&S
CXR and sweat test
what is transient tachypnoea of the newborn?
commonest cause of resp distress in the newborn period
caused by delayed resorption of fluids in the lungs
most common after C-sections
CXR- hyperinflation of lungs and fluid in the horizontal fissure
what causes acute epiglottitis?
Haemophilus influenza type B
incidence decreased since vaccination
features of acute epiglottitis?
rapid onset
high temp, generally unwell
stridor
drooling of saliva
mx of acute epiglottitis?
do NOT examine throat- can cause airway obstruction
call anaesthetist to intubate
IV cefuroxime
cause of whooping cough?
Bordetella pertussis (gram negative bacteria)
features of whooping cough?
should be suspected in a person who has an acute cough >14 days without apparent cause, and has one or more of the following features:
- paroxysmal cough- worse at night and after feeding
- inspiratory whoop
- post-tussive vomiting
- undiagnostic apnoeic attacks in young infants
diagnosis of whooping cough?
per nasal swab culture
PCR and serology
admit if under 6 months
mx of whooping cough?
oral macrolide e.g. clarithromycin if the cough is within prev 21 days
household contacts offered Abx prophylaxis
School exclusion- 48 hours after commencing Abx
NOTIFIABLE DISEASE
When are congenital heart diseases usually picked up?
antenatal 20 weeks gestation- foetal echo
left to right shunts?
breathlessness, asymptomatic
VSD
ASD
PDA
right to left shunts?
cyanotic TOF TPGA Coarctation of aorta Hypoplastic left heart syndrome
causes of CHDs?
maternal rubella,SLE, Diabetes
warfarin
foetal alcohol syndrome
downs, pataus, turners, Edwards syndromes
what happens to the foetal circulation pre and post delivery?
foramen ovale between atria and ductus arteriosus between pulmonary artery and aorta (to bypass lungs)
blood flows R -> L
at birth, first breath increases pulmonary blood flow and LA pressure, no placenta decreases RA pressure so causes foramen ovale to close
first hour/days of life- ductus arteriosus closes
what are the innocent murmurs?
Venous hums and Still's murmur 1. aSymptomatic murmur 2. Soft blowing murmur- venous hum 3. Systolic murmur only 4. Left sternal edge- Still's murmur changes with position
what causes a venous hum?
turbulent blood flow returning to the heart
heard as a continuous blowing noise just below the clavicles
what is a still’s murmur heard?
low-pitched sounds heard best at left sternal edge
features of a PDA?
Associated with prematurity
continuous machinery murmur beneath the clavicles
mx- Indomethacin (NSAID) closes the correction in the majority of cases
prostaglandin is useful to keep the duct open until after surgical repair
4 features of tetralogy of fallot?
- VSD
- RVH
- Pulmonary stenosis
- Overriding aorta
CXR- boot-shaped heart, ECG shows RVH
Mx- surgical repair
when does transposition of the great arteries classically present?
on day 2 when ductus arteriosus opens
presentation of heart failure in infants?
SOB worse on exertion e.g. feeding, sweating, poor feeding and recurrent chest infections
what is respiratory distress syndrome?
causes by a deficiency in surfactant (produced by type 2 pneumocytes in the alveolar epithelium) which lowers surface tension and leads to widespread alveolar collapse and inadequate gas exchange
RF for RDS?
Prematurity
male sex
diabetic mothers
features of RDS?
Tachypnoea laboured breathing chest wall recession, chest hyperinflation nasal flaring expiratory grunt tracheal tug decreased feeds
CXR of RDS?
ground glass appearance
mx of RDS?
maternal steroids during pregnancy for prevention
O2
assisted ventilation
exogenous surfactant given via ET tube
referral points in terms of child development for smiling, babbling, 1-2 words, sitting unsupported and walking?
no smiling at 10 weeks no sitting unsupported at 12 months no walking at 18 months no babbling at 8 months no words after 2 years
causes of developmental delay?
- genetics
- pregnancy- congenital infections, drugs and alcohol exposure
- birth- prematurity, birth asphyxia
- childhood- meningitis, neglect/abuse, hearing/visual impairment, NAI
what is Prader-Willi syndrome?
gene deleted from Chr 15
if father- Prada willi
if mother- Angelman
causes hypotonia during infancy, short stature, hypogonadism, childhood obesity, learning difficulties
signs of down’s syndrome?
Trisomy 21
Umbilical hernia, macroglossia, hypotonia, short stature, congenital heart disease, epicanthic folds, bradycephaly, single palmar crease, flattened nose
mx of Down’s syndrome?
ECHO at birth
regular hearing, visual, dental follow up
coeliac screening
later complications of down’s syndrome?
subfertility learning difficulties short stature resp infections ALL hypothyroidism Alzheimers
what is cerebral palsy?
a permanent disorder of movement and posture to a non-progressive lesion of motor pathways in the developing brain
causes of CP?
80% is antenatal- CV haemorrhage or ischaemia, congenital infection, structural maldevelopment
10% is hypoxic-ischaemic- injury
10% is post-natal- periventricular leukomalacia, meningitis/encephalitis etc
types of CP?
Spastic (80%)- UMN lesion, hypertonia, rigidity, hyperreflexia (quadriplegic, hemiplegic etc)
Athetoid- affects basal ganglia
Ataxic- poor concentration, cerebellar lesions, ataxic gait, intention tremor
Mixed
mx of spasticity of CP?
oral diazepam
oral and intrathecal baclofen
botox
orthopaedic surgery
types of seizures in childhood?
JME
Infantile spasms (west syndrome)
Absence seizures
Febrile Convulsions
features of JME?
classic history is throwing drinks or cereal about in the morning
mx of JME?
sodium valproate or lamotrigine
SEs of sodium valproate?
Teratogenic, so patients need careful advice about contraception
Liver damage and hepatitis
Hair loss
Tremor
what is west syndrome?
brief spasms beginning in first few months of life
1. flexion of head, trunk, limbs -> extension of arms
lasts 1-2 secs
2. progressive mental handicap
3. EEG
poor prognosis
tx of absence seizures?
sodium valproate
mx of seizures in a child?
ABC, O2, check BG, IV access, time 5 mins- IV lorazepam or buccal midazolam 15 mins- repeat lorazepam 20 mins- phenytoin over 20 mins (call for help) 40 mins- rapid sequence induction
what is ADHD?
Inattention and/or hyperactivity/impulsivity that is persistent
mx of ADHD?
10 week initial ‘watch and wait’ approach
1st line- methylphenidate (CNS stimulant)
SE- abdo pain, nausea, dyspepsia, cardio toxic
need baseline ECG
what is osteogenesis imperfecta?
a group of disorders of collagen metabolism resulting in bone fragility and fractures
AD inheritance
features of OI?
fractures following minor trauma
blue sclera
deafness secondary to otosclerosis
dental imperfections are common
Ix of OI?
bloods- FBC, U&E, CRP, Vit D, Ca, PTH XR Skeletal survey ECHO MRI if suspect tumour
causes of jaundice in the first 24 hours?
ALWAYS PATHOLOGICAL rhesus haemolytic disease ABO haemolytic disease hereditary spherocytosis G6PD deficiency neonatal sepsis
what causes jaundice from 24 hours to 2 weeks?
due to increase RBC conc and decreased half life-physiological
causes of prolonged jaundice?
biliary atresia (needs surgical intervention), hypothyroidism, UTI, breast milk jaundice, TORCH infection
what is conjugated bilirubin?
Unconjugated bilirubin is conjugated in the liver. Conjugated bilirubin is excreted in two ways: via the biliary system into the gastrointestinal tract and via the urine.
what causes jaundice in the premature neonate?
In premature babies, the process of physiological jaundice is exaggerated due to the immature liver.
Ix of prolonged jaundice?
Full blood count and blood film for polycythaemia or anaemia
Conjugated bilirubin: elevated levels indicate a hepatobiliary cause
Blood type testing of mother and baby for ABO or rhesus incompatibility
Direct Coombs Test (direct antiglobulin test) for haemolysis
Thyroid function, particularly for hypothyroid
Blood and urine cultures if infection is suspected. Suspected sepsis needs treatment with antibiotics.
Glucose-6-phosphate-dehydrogenase (G6PD) levels for G6PD deficiency
complications of jaundice?
kernicterus- encephalopathy resulting from deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei
features of kernicterus?
sleepiness poor feeding poor muscle tone high-pitched cry irritability seizures arched back
mx of jaundice?
phototherapy- converts unconjugated bilirubin into harmless water-soluble metabolites-> excreted in bile and urine
exchange transfusion
causes of congenital hypothyroidism?
seen in heel prick test
- maldescent of the thyroid and athyrosis
- dyshormogenesis
- iodine deficiency
- hypothyroidism due to TSH deficiency
what is congenital adrenal hyperplasia?
21-hydroxylase deficiency- deficiency of steroids and aldosterone
high levels of ACTH due to low steroid level
high ACTH can produce adrenal androgens that may virilise a female patient
presentation of CAH in males and females?
females- virilisation of external genitalia
males- salt loss (80%) or tall stature and precocious puberty
salt loss= metabolic acidosis, vomiting, dehydration, weight loss, floppiness
what do glucocorticoid hormones do?
act to help the body deal with stress, raise blood glucose, reduce inflammation and suppress the immune system
mx of salt losing crisis?
IV sodium chloride, hydrocortisone, fludrocortisone
what is the definition of precocious puberty?
development of secondary sexual characteristics before 8 years in females and 9 years in males
what is ‘true’ causes of precocious puberty?
premature activation of HPO axis
in males- CNS lesions, HCG secretion, hepatoblastoma, primary hypothyroidism
what is androgen insensitivity syndrome?
an x-linked condition due to end-organ resistance to testosterone causing males to have a female phenotype
causes of non-blanching purpuric rash?
meningococcal, HSP, enterovirus, thrombocytopenia
key consequences of CF mutation?
Thick pancreatic and biliary secretions that cause blockage of the ducts, resulting in a lack of digestive enzymes such as pancreatic lipase in the digestive tract
Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections
Congenital bilateral absence of the vas deferens in males. Patients generally have healthy sperm, but the sperm have no way of getting from the testes to the ejaculate, resulting in male infertility
