What Is/Are..... Flashcards
Possible consequences of NEC
Strictures, with incr risk of obstruction
Malabsorption if substantial resection was required
TPN complications:
Thrombophlebitis, central line infection, hyperglycaemia, cirrhosis, metabolic imbalance, acidosis, osteopenia, cholestasis
Respiratory Distress Syndrome
RDS aka Hyaline Membrane Disease
Absence of surfactant in lungs at birth due to prematurity
Type 2 pneumocytes begin to produce surfactant at 22wks
Surfactant reduces surface tension in the alveoli preventing them from collapsing, it also enhances gas exchange
Complications of RDS
Pneumothorax: high pressure O -> alveoli-> interstitium
Lobar collapse
Chronic lung disease of prematurity: pressure and volume trauma caused by long term ventilation
Cor pulmonale: due to pulm htn
Intra ventricular haemorrhage: due to fluctuations in BP and pH
Diaphragmatic hernia
Abdominal contents herniate up into the thorax (L side)
Usually detected on antenatal USS
Mass effects during development -> pulmonary hypoplasia
Asymmetrical chest signs & CXR
NG tube -> stabilise -> surgical repair
Vigorous resus -> pneumothorax
Tracheo-oesophageal fistula
Failure of normal development
Abnormal connection between trachea and oesophagus
Frothing at mouth seen as infant unable to swallow saliva
May lead to aspiration pneumonia
Laryngomalacia
Congenital laryngeal stridor
Flaccid supraglottic structures
Presents later than RDS
Most severe in older babies
Nitrogen Washout Test
Differentiates betw resp and cardiac causes of cyanosis
10 mins 100% Oxygen = max saturation of pulm circulation
Cyanosis improves: resp cause
No improvement: R -> L shunt
VATER / VACTERL
Vertebral anomalies, Anal malformations, Tracheoesophogeal-oesophageal fistula, Radial and Renal abnormalities
+ Cardiac deformity
+ Limb deformity
Three or more req to apply the term
Total Parenteral Nutrition
Elemental feed delivered through a catheter into a central vein
When enteral feeding is inappropriate because:
gut needs to be rested
absorption is inadequate
following severe trauma/burns
Patent Ductus Arteriosus
Failure of ductus between aorta and pulmonary artery to close
Left to right shunt
Bounding pulse, systolic murmur
If more severe -> HF : desaturation and respiratory distress
If symptomatic -> indomethacin or surgical ligation
Tetralogy of Fallot
An overriding aorta
Right ventricular outflow tract obstruction
Ventricular septal defect
Right ventricular hypertrophy
Assoc with: Noonan’s, Down’s,
Congenital heart disease:
L -> R shunt
Breathless
Ventricular Septal Defect
Persistent Ductus Arteriosus
Atrial Septal Defect
Congenital heart disease:
R -> L shunt
Blue
Tetralogy of Fallot
Transposition of the Great Arteries
Congenital heart disease:
Mixed
Breathless and Blue
Atrioventricular septal defect
Congenital heart disease:
Outflow obstruction in well child
Asymptomatic with murmur
Pulmonary stenosis
Aortic stenosis
Congenital heart disease:
Outflow obstruction in sick neonate
Collapsed with shock
Coarctation of the aorta
Hypo plastic left heart syndrome
Innocent murmurs
aSymptomatic
Soft blowing murmur
Systolic murmur only
left Sternal edge
No parasternal thrill
No radiation
Physiological jaundice
>50% term babies, >80% preterm babies Mild Day 2-3 until end of first week Inefficient bilirubin metabolism Immature hepatic function Frequent RBC breakdown due to short 70 day lifespan
Breast milk jaundice
Diagnosis of exclusion
Appears within first 2 weeks, may last weeks
Mild, self limiting
? Dehydration due to poor feeding technique
? Component of breast milk inhibiting bilirubin metabolism
Kernicterus
Too much unconjugated bilirubin
Fat solubility -> crosses blood brain barrier
Deposited in: basal ganglia and brain stem nuclei
Lethargy, poor feeding, irritability, incr tone, seizures, coma
Less common due to prevention of resus haemolytic disease w. anti-D
Jaundice
Rhesus haemolytic disease ABO incompatibility G6PD deficiency Spherocytosis Congenital infection
Jaundice at 2 days - 2 weeks
Physiological jaundice
Breast milk jaundice
Dehydration
Infection
Rhesus haemolytic disease
Usually identified antenatally Severe presentation: anaemia, hydrops, hepatosplenomegaly D Kell Duffy
ABO incompatibility
ABO antibodies = mostly IgM don’t cross placenta
Gp O women may have IgG anti A/B haemolysin
Peak jaundice at 12-72 hrs
Positive direct antibody (Coombs) test
G6PD deficiency
Mediterranean/Middle East/Far East/Afro-Caribbean descent X linked Haemolysis precipitated by: Infection Drugs: ? Favs beans
Phototherapy
450nm light (blue-green visible spectrum)
Photoisomerizes unconjugated BR -> water soluble
Excreted in urine
Cover eyes
Regular serum BR
Hypoxic-Ischaemic Encephalopathy
Perinatal hypoxia
Clinical manifestations can present up to 48hr post asphyxia
Neuronal damage: primary death, delayed reperfusion secondary death
Delay allows neuroprotection using hypothermia
Caput succedaneum
Bruising + oedema of presenting part
Extends beyond margins skull bones
Resolves in a few days
Cephalhaematoma
Bleeding below periosteum
Haematoma confined by skull suture margins, soft centre
Often involves parietal bone
Resolves over several weeks
Chignon
Oedema and bruising from Ventouse delivery
Subaponeurotic haemorrhage
Diffuse boggy swelling of scalp
V uncommon
–>severe blood loss, hypovolaemic shock and coagulopathy
Brachial nerve palsy
Traction to brachial plexus nerve roots
Breech delivery or shoulder dystocia
C5+6 = Erb’s palsy +/- phrenic nerve palsy (elevated diaphragm)
If not resolved by 2-3 months -> ortho/plastics
Intraventricular haemorrhage
In 25% V low birthweight infants Diagnosed on cranial ultrasound First 72 hrs Germinal matrix above caudate nucleus Assoc with perinatal asphyxia and RDS May impair CSF drainage -> vent dilatation -> hydrocephalus
Retinopathy of prematurity
Vascular proliferation at junction betw vasc + non vasc retina
–> retinal detachment –> fibrosis –> blindness
Oxygen therapy
35% V low birthweight infants
Screened weekly by opthalmology
Laser therapy reduces visual impairment
Bronchopulmonary Dysplasia
Oxygen requirement at 36 wks post-menstrual age
Due to pressure and volume trauma
From artificial ventilation, oxygen toxicity, infection
Transient tachypnoea of the newborn
Diagnosis of exclusion
Delay in resorption of lung liquid
Most common cause of resp distress, more post c-section
Additional ambient oxygen, may take a few days to resolve
CXR: fluid in horizontal fissure
Meconium aspiration
Increased with incr gestation
Lung irritant -> mech obstruction + chemical pneumonitis
Artificial ventilation
CXR: overinflation, patches of collapse + consolidation
Air leak –> pneumothorax/pneumomediastinum
Persistent pul htn -> inadequate oxygenation despite ventilation
Neonatal pneumonia
PROM
Chorioamnionitis
Low birthweight
Persistent pulmonary hypertension of the newborn
High pulm vasc resistance: R -> L shunt Cyanosis soon after birth Mech vent + circulatory support Inhaled NO beneficial CXR: normal heart size, pulm oligaemia Urgent echo to rule out congenital heart disease
Early onset sepsis
PROM, chorioamnionitis
Resp distress, apnoea, T instability
Broad spec IV abx:
Gp B strep, gram +ve, gram -ve: benzylpenicillin + gentamycin
Blood cultures: if -ve with no clinical signs stop abx after 48hrs
LP: if neuro signs/+ve blood cultures
Congenital Adrenal Hyperplasia
AR
absence 21 hydroxylase
Systemic Juvenile Idiopathic Arthritis - Still’s disease
High fever, nocturnal spikes Malaise + anorexia Salmon pink rash Anaemia Raised platelets + neutrophils Raise CRP
Kawasaki’s
Vasculitis
Bacterial toxin acts as super-antigen
6 months - 4 yrs
Prolonged fever, cervical lymphadenopathy, strawberry tongue, conjunctival infection, rash, arthralhia, finger tip desquamation
Coronary artery involvement -> aneurysm or thrombosis
Contraindications to LP
Resp compromise Cardiac compromise Raised ICP Coma/rapid decr in consciousness Focal neuro signs Coag disorder or thrombocytopenia Local infection at LP site
UTI: causative organisms
E.coli Proteus: common in boys Pseudomonas: with urinary tract abnormality Klebsiella Strep.faecalis
Vesicoureteric reflux
Ureters enter bladder at perpendicular angle
= shorter intramural course
-> reflux of urine on bladder contraction
Severe reflux: renal pelvis dilatation, calyces
Incomplete bladder emptying: predisposition to infection
Infection -> scarring, serious bilat scarring ->chronic renal failure
Pyloric stenosis
Projectile vomiting 4 - 8 wks M-4:F-1 1/500 males Incr risk with family history Rapid dehydration: untreated -> death
Gastro-oesophageal reflux
Severe vomiting
0 - 3 months
Full abdomen
Worsens over weeks
Posseting
Normal
Follows feeding
Non-forceful regurgitation of milk
Signs of hypovolaemic shock
Incr HR
Decr BP
Poor perfusion: slow CRT, cool peripheries
Signs of cellular dehydration
Decr tissue turgor
Sunken eyes
Depressed ant fontanelle
Signs of mild dehydration
Dry mucous membranes
Dry nappies
Signs of raised intracranial pressure
Bulging, tense anterior fontanelle Focal neuro signs Unequal pupils Htn + low HR: cushing's reflex Papilloedema
Signs of neck stiffness
Opisthotonos: lying with arched back
Brudzinski’s sign:
flexion of neck when lying supine -> flexion of hips + knees
Kernig’s sign:
extension of knees when supine with flexed hips + knees -> back pain
Meningitis causing pathogens:
Infants
Gp B strep
E.coli and other G-ves
Listeria
Meningitis causing pathogens:
Children 1 month - 6 yrs
Neisseria meningitidis
Haemophilus influenzae
Streptococcus pneumoniae
Meningitis causing pathogens:
Children > 6 yrs
Neisseria meningitidis
Streptococcus pneumoniae
Complications of meningitis
Deafness: inflammatory damage to hair cells
Cranial nerve palsies: local vasculitis
Cerebral abscesses: raise ICP, require surgical drainage
Epilepsy: abscess/ local infarction
Hydrocephalus: inflammation/exudate block reabsorption of CSF
Mental impairment: more common with pneumococcal meningitis
CSF features of bacterial meningitis
Turbid appearance
Polymorphs ++
Protein: >1.5 g/L
Glucose:
CSF features of viral meningitis
Clear appearance
Some lymphocytes
Protein: 66% plasma glucose
CSF features of TB meningitis
Fibrin web visible
Some lymphocytes
Protein: 1-5 g/L
Glucose
Trisomy 13
1/14000
Patau's syndrome IUGR Poldactyly Cleft lip + palate Scalp, cardiac + renal abnormalities
Trisomy 18
1/8000
Edward's syndrome IUGR Flexed overlapping fingers Rocker bottom feet Cardiac + renal abnormalities
Trisomy 21
1/700
Developmental delay + mod-sev learning disabilities, Avg IQ 50
Round face, epicanthic folds, protruding tongue, hypotonia, single palmar crease, sandle-gap toes, flat occipitut
Duodenal + biliary atresia, VSD + ASD
XXY
1/1000 male
Klinefelter's syndrome Tall Female pattern body fat + hair growth Gynaecomastia Small testicles Mild-mod learning disabilities Incr risk osteoporosis + breast cancer
X0
1/2500 female
Turner's syndrome Short stature + wide carrying angle Widely spaced nipples Normal intelligence Assoc w. Coarctation of the aorta Webbed neck, lymphoedema of hands + feet Amenorrhoea Treat with GH and oestrogen
Fragile X syndrome
1/4000
Most common cause of intellectual impairment in men
X linked dominant triple repeat: fmr1
Expansion of CGG at xq27.3 to > 200 repeats
= severe learning disabilities + autistic features
High forehead, large ears, long face, prominent jaw
Fetal alcohol syndrome
Growth restriction
Cardiac + GU abnormalities
Intellectual impairment + emotional disturbance
Intrauterine disruption
Severe oligohydramnios: compression induced deformity e.g. contractures
Amniotic bands: limb reduction deformities + lateral cleft lips
Health problems assoc w. Down’s syndrome
Structural abnormalities, congenital heart disease, duodenal + biliary atresia Sinusitis + otitis media -> conductive hearing problems Hypothyroidism Cataracts Acquired heart disease Incr risk: AML, testicular cancer Early onset Alzheimer's Atlanto-axial instability
Cri du chat
Deletion: end of Chr 5
Severe mental retardation
High pitched cry
Wolf-hirschhorn
Deletion: end of Chr 4
Severe mental retardation
Failure to thrive
William’s
Micro deletion: 7q11
Peri orbital fullness, everted lower lip, full checks, developmental delay, engaging personality, heart defects
Prader-Willi
Microdeletion: Chr15q11-13 from father
Initially: hypotonia + poor feeding
Later: insatiable appetite, obesity, mental retardation
Angelman
Microdeletion: Chr15 q11-13 from mother Inappropriate laughter Convulsions Ataxia Flapping Mental retardation
DiGeorge
Microdeletion: Chr22 q11-12
Cardiac defects, absent thymus, under active parathyroid glands
Anaemia, immune deficiency, hypoglycaemia, seizures
Cleft palate, cognitive defects
Screening for Down’s
Nuchal translucency at 11-14wk dating scan
Triple serum test at 15 wks:
Oestradiol, BetaHCG: incr, Alpha-fetoprotein: decr
Probability of Down’s calculated
High risk: amniocentesis from15 wks, chorionic villus sampling from 11wk
0.5-2% risk spontaneous miscarriage
Termination offered
Risk of obstruction with inguinal hernia
6 month old: >50-60%
1year old: >10%
2 year old: >2%
Indirect inguinal hernia
1/50 boys
Due to patent processus vaginalis
Usually right sided
More common in premature infants
Genetic causes trisomy 21
94% meiotic non-dysjunction
5 % Robertsonian translocation
1% Mosaicism
Tay Sachs
Deaf
Blind
Repeated infections
Screening for Down’s syndrome
11-14 wk dating scan: nuchal translucency
15 wk triple serum test: oestriol, beta- hCG (incr), alpha-fetoprotein (decr)
= probability of Down’s
Diagnostic tests:
Amniocentesis >15 wks, chorionic villus sampling >11wks
0.5-2% risk of miscarriage
Option to terminate upon diagnosis
What is Osgood-Schlatter disease?
10-15 yrs 1/100000
traction apophysitis
(cartilage detachment from tibial tuberosity)
due to repeated avulsion during growth spurt i.e. overuse
usually a lump over the tibial tubercle
self limiting
What is slipped femoral epiphysis?
overweight boys 10-15 yrs
1/50000
FE displaced posterolaterally following minor trauma/spontaneously
bilateral in 1/5
What is Perthe’s disease?
avascular necrosis of the femoral head boys 5-10 yrs limp develops over 1 month may be preceded by infection/trauma over 18-36 months revacularisation and reossification occurs may be fhx, 1/5 bilateral more common in SCD
Osteogenic sarcoma
girls 10-14 yrs boys 15-18 yrs 1/100000 persistent pain in long bones of leg P53 mutation + Li Fraumeni synd predispose
Ewing’s sarcoma
5-20yrs
1000000
local disease 60-70% survival
metastatic disease 20-30%
What are the complications of corrective surgery for a slipped femoral epiphysis?
avascular necrosis
fusion in the new position, slight coax vara deformity
chondrolysis -> pain + arthritis
Systemic Juvenile Idiopathic Arthritis
1-4 yrs 1/100000 fever rash hepatomegaly usually self limiting polyarticular > 4 joints pauciarticular 4 or less systemic needs to be present for 3 months to make the diagnosis
What is Septic Arthritis?
recent URTI strep pyogenes haemophilus influezae strep pneumoniae staph aureas
What is Transient Synovitis?
2-12 yrs common often initiated by viral illness may have fever and incr acute phase reactants spontaneous resolution
Developmental hip dysplasia
at birth
‘clicky hips’ on Ortolani’s and Barlow’s manoeuvres
6-10/100 live births
What do different immune deficiencies predispose to?
immunoglobulin deficiency (agammaglobulinaemia)-> severe bacterial infections complement disorder -> encapsulated organisms and AI disorder phagocyte dysfunction (chronic granulomatous disease) -> severe bacterial and fungal infection
What is Severe Combined Immune Deficiency?
usually X linked inherited
affects humoral and cellular components
-> early, severe, atypical infections
only possible cure - BM transplant
How many resp tract infections per year is normal
preschool age 6-8 is normal
most will be self limiting viral infections
What causes secondary immune deficiency?
drugs
infection
malignancy
What are the causes of HIV in children?
vertical transmission:
in utero, during delivery, breast feeding
infected blood products
dirty needles
What are AIDS defining illnesses?
TB
candidiasis
What are common presentations of HIV infection?
mild immunosuppression: lymphadenopathy, parotitis, persistent diarrhoea, recurrent bacterial infection
moderate immunosuppression:
failure to thrive, candidiasis, lymphocytic interstitial pneumonitis, opportunistic infections, focal organ disease
How do you manage HIV?
antiretroviral therapy
starting regimens incl:
2 nucleoside analogue reverse transcriptase inhibitors (zidovudine, zalcitabine) with a non nucleoside reverse transcriptase inhibitor (nevirapine) or a protease inhibitor (ritonavir)
What is the toxic dose of paracetamol?
> 130mg/kg
When is gastric lavage used for paracetamol OD?
if patient presents within 1 hr with potentially life-threatening OD
risks incl: aspiration, GI perforation or haemorrhage
never perform with reduced consciousness unless intubated
When might activated charcoal be used for OD?
if presentation is within 1 hr of ingestion or 2hrs of a drug known to delay gastric emptying
given orally or by NG tube
How to manage paracetamol OD acutely
blood paracetamol level at 4 hrs post ingestion
plot on treatment graph
if in toxic range give N-acteylcysteine
if presentation >8hr post injestion start NAC immed and send bloods
if level not toxic OR if INR, ABG + renal function are normal and level is 24 hrs NAC use depends on RF
What puts patients in high risk category after paracetamol OD
chronic alcohol misuse malnutrition/anorexia pre-existing liver disease HIV +ve on liver enzyme inducing drugs e.g. carbamazepine, phenytoin, phenobarbitone, rifampicin
Managing OD
admit all paediatric self harm / attempted suicide
Modifiable risk factors of sudden infant death syndrome?
Sleeping position: on back
Smoking: avoid maternal + paternal smoking
16-20 degree room temp, don’t tuck blanket higher than shoulders
Breastfeed
Rosving’s sign
Pain in RIF in response to left sided palpation
Suggests peritoneal irritation in the RIF
McBurney’s point
1/3 of distance between ASIS and umbilicus
Typically tenderness and guarding is seen over this point in appendicitis
Cough sign
Pain in RIF after a voluntary cough
Suggests peritoneal irritation in the RIF
Obturator sign
Pain on internal rotation of flexed right thigh
Caused by inflammatory mass overlying the psoas muscle
MCADD
Medium Chain Acetyl co-a Dehydrogenase Deficiency
Inborn error of metabolism
Fat can’t be broken down
Low energy + hypoglycaemic episodes
Ddx chronic cough
asthma, inhaled foreign body, sinusitis, post nasal drip, habit cough, allergic rhinitis, TB, CF, bronchiectasis, immune deficiency
Bone age
A test used in assessment of short stature
Age is defined from amt of ossification seen in their bones
Radiographs of carpal bones are used
Each of the centres of ossification is scored and converted into bone age
Transient tachypnoea of the newborn (TTN)
Due to delayed clearance of fluid from fetal lungs
More common in babies born by c section
Incr adrenaline -> active uptake of fetal lung fluid by sodium channels
Rr can be 100-120
CXR to rule out congenital pneumonia
Will show hyperinflation, oedema + fluid
Oxygen + empirical abx as initially sepsis cannot be ruled out
Prehn’s sign
Tenderness relieved by elevating the scrotum
+ve indicates epididymitis
Bloom’s syndrome
AR Short stature Facial photosensitive rash Narrow face Subfertility Immune deficiency
The most common causes of ophthalmia neonatorum
I.e. Conjunctivitis in first few wks of life Staph aureas Chlamydia trachomatis Neisseria gonorrhoea Chemical irritation (silver nitrate)
Indications of NAI
Discrepancy between presentation and history given
Change in history over time/ between different people
Delay in presentation
Unusual reaction to the injury
Repeated injuries
Hx of NAI/ suspicious injury in sibling
Other signs of neglect/FTT
Barlow’s test
Neonatal screening for DDH
Hip can be easily displaced posterior lay out of acetabulum
On adduction of leg with post pressure
Ortolani’s test
Neonatal screening for DDH
Femoral head can be reduced back into acetabulum
On abduction of leg with anterior pressure
Hand foot syndrome?
Children with SCD
Present with tender swelling of hand/wrist/feet
Precipitated by stress or cold
Schilling test
IM vit B12
Radioactive cobalt labelled oral vit B12 given
If excreted in urine = normal
If not: oral radiolabelled b12 given with IF
If not radiolabelled b12 excreted pernicious anaemia confirmed
Still no excretion = bowel related malabsorption
