What Is/Are..... Flashcards
1
Q
Possible consequences of NEC
A
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
2
Q
Respiratory Distress Syndrome
A
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
3
Q
Complications of RDS
A
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
4
Q
Diaphragmatic hernia
A
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
5
Q
Tracheo-oesophageal fistula
A
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
6
Q
Laryngomalacia
A
Congenital laryngeal stridor
Flaccid supraglottic structures
Presents later than RDS
Most severe in older babies
7
Q
Nitrogen Washout Test
A
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
8
Q
VATER / VACTERL
A
Vertebral anomalies, Anal malformations, Tracheoesophogeal-oesophageal fistula, Radial and Renal abnormalities
+ Cardiac deformity
+ Limb deformity
Three or more req to apply the term
9
Q
Total Parenteral Nutrition
A
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
10
Q
Patent Ductus Arteriosus
A
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
11
Q
Tetralogy of Fallot
A
An overriding aorta
Right ventricular outflow tract obstruction
Ventricular septal defect
Right ventricular hypertrophy
Assoc with: Noonan’s, Down’s,
12
Q
Congenital heart disease:
L -> R shunt
Breathless
A
Ventricular Septal Defect
Persistent Ductus Arteriosus
Atrial Septal Defect
13
Q
Congenital heart disease:
R -> L shunt
Blue
A
Tetralogy of Fallot
Transposition of the Great Arteries
14
Q
Congenital heart disease:
Mixed
Breathless and Blue
A
Atrioventricular septal defect
15
Q
Congenital heart disease:
Outflow obstruction in well child
Asymptomatic with murmur
A
Pulmonary stenosis
Aortic stenosis
16
Q
Congenital heart disease:
Outflow obstruction in sick neonate
Collapsed with shock
A
Coarctation of the aorta
Hypo plastic left heart syndrome
17
Q
Innocent murmurs
A
aSymptomatic
Soft blowing murmur
Systolic murmur only
left Sternal edge
No parasternal thrill
No radiation
18
Q
Physiological jaundice
A
>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
19
Q
Breast milk jaundice
A
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
20
Q
Kernicterus
A
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
21
Q
Jaundice
A
Rhesus haemolytic disease ABO incompatibility G6PD deficiency Spherocytosis Congenital infection
22
Q
Jaundice at 2 days - 2 weeks
A
Physiological jaundice
Breast milk jaundice
Dehydration
Infection
23
Q
Rhesus haemolytic disease
A
Usually identified antenatally Severe presentation: anaemia, hydrops, hepatosplenomegaly D Kell Duffy
24
Q
ABO incompatibility
A
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
25
G6PD deficiency
```
Mediterranean/Middle East/Far East/Afro-Caribbean descent
X linked
Haemolysis precipitated by:
Infection
Drugs: ?
Favs beans
```
26
Phototherapy
450nm light (blue-green visible spectrum)
Photoisomerizes unconjugated BR -> water soluble
Excreted in urine
Cover eyes
Regular serum BR
27
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
28
Caput succedaneum
Bruising + oedema of presenting part
Extends beyond margins skull bones
Resolves in a few days
29
Cephalhaematoma
Bleeding below periosteum
Haematoma confined by skull suture margins, soft centre
Often involves parietal bone
Resolves over several weeks
30
Chignon
Oedema and bruising from Ventouse delivery
31
Subaponeurotic haemorrhage
Diffuse boggy swelling of scalp
V uncommon
-->severe blood loss, hypovolaemic shock and coagulopathy
32
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
33
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
```
34
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
35
Bronchopulmonary Dysplasia
Oxygen requirement at 36 wks post-menstrual age
Due to pressure and volume trauma
From artificial ventilation, oxygen toxicity, infection
36
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
37
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
38
Neonatal pneumonia
PROM
Chorioamnionitis
Low birthweight
39
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
```
40
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
41
Congenital Adrenal Hyperplasia
AR
| absence 21 hydroxylase
42
Systemic Juvenile Idiopathic Arthritis - Still's disease
```
High fever, nocturnal spikes
Malaise + anorexia
Salmon pink rash
Anaemia
Raised platelets + neutrophils
Raise CRP
```
43
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
44
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
```
45
UTI: causative organisms
```
E.coli
Proteus: common in boys
Pseudomonas: with urinary tract abnormality
Klebsiella
Strep.faecalis
```
46
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
47
Pyloric stenosis
```
Projectile vomiting
4 - 8 wks
M-4:F-1
1/500 males
Incr risk with family history
Rapid dehydration: untreated -> death
```
48
Gastro-oesophageal reflux
Severe vomiting
0 - 3 months
Full abdomen
Worsens over weeks
49
Posseting
Normal
Follows feeding
Non-forceful regurgitation of milk
50
Signs of hypovolaemic shock
Incr HR
Decr BP
Poor perfusion: slow CRT, cool peripheries
51
Signs of cellular dehydration
Decr tissue turgor
Sunken eyes
Depressed ant fontanelle
52
Signs of mild dehydration
Dry mucous membranes
| Dry nappies
53
Signs of raised intracranial pressure
```
Bulging, tense anterior fontanelle
Focal neuro signs
Unequal pupils
Htn + low HR: cushing's reflex
Papilloedema
```
54
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
55
Meningitis causing pathogens:
Infants
Gp B strep
E.coli and other G-ves
Listeria
56
Meningitis causing pathogens:
Children 1 month - 6 yrs
Neisseria meningitidis
Haemophilus influenzae
Streptococcus pneumoniae
57
Meningitis causing pathogens:
Children > 6 yrs
Neisseria meningitidis
| Streptococcus pneumoniae
58
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
59
CSF features of bacterial meningitis
Turbid appearance
Polymorphs ++
Protein: >1.5 g/L
Glucose:
60
CSF features of viral meningitis
Clear appearance
Some lymphocytes
Protein: 66% plasma glucose
61
CSF features of TB meningitis
Fibrin web visible
Some lymphocytes
Protein: 1-5 g/L
Glucose
62
Trisomy 13
1/14000
```
Patau's syndrome
IUGR
Poldactyly
Cleft lip + palate
Scalp, cardiac + renal abnormalities
```
63
Trisomy 18
1/8000
```
Edward's syndrome
IUGR
Flexed overlapping fingers
Rocker bottom feet
Cardiac + renal abnormalities
```
64
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
65
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
```
66
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
```
67
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
68
Fetal alcohol syndrome
Growth restriction
Cardiac + GU abnormalities
Intellectual impairment + emotional disturbance
69
Intrauterine disruption
Severe oligohydramnios: compression induced deformity e.g. contractures
Amniotic bands: limb reduction deformities + lateral cleft lips
70
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
```
71
Cri du chat
Deletion: end of Chr 5
Severe mental retardation
High pitched cry
72
Wolf-hirschhorn
Deletion: end of Chr 4
Severe mental retardation
Failure to thrive
73
William's
Micro deletion: 7q11
| Peri orbital fullness, everted lower lip, full checks, developmental delay, engaging personality, heart defects
74
Prader-Willi
Microdeletion: Chr15q11-13 from father
Initially: hypotonia + poor feeding
Later: insatiable appetite, obesity, mental retardation
75
Angelman
```
Microdeletion: Chr15 q11-13 from mother
Inappropriate laughter
Convulsions
Ataxia
Flapping
Mental retardation
```
76
DiGeorge
Microdeletion: Chr22 q11-12
Cardiac defects, absent thymus, under active parathyroid glands
Anaemia, immune deficiency, hypoglycaemia, seizures
Cleft palate, cognitive defects
77
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
78
Risk of obstruction with inguinal hernia
6 month old: >50-60%
1year old: >10%
2 year old: >2%
79
Indirect inguinal hernia
1/50 boys
Due to patent processus vaginalis
Usually right sided
More common in premature infants
80
Genetic causes trisomy 21
94% meiotic non-dysjunction
5 % Robertsonian translocation
1% Mosaicism
81
Tay Sachs
Deaf
Blind
Repeated infections
82
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
83
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
84
What is slipped femoral epiphysis?
overweight boys 10-15 yrs
1/50000
FE displaced posterolaterally following minor trauma/spontaneously
bilateral in 1/5
85
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
```
86
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
```
87
Ewing's sarcoma
5-20yrs
1000000
local disease 60-70% survival
metastatic disease 20-30%
88
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
89
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
```
90
What is Septic Arthritis?
```
recent URTI
strep pyogenes
haemophilus influezae
strep pneumoniae
staph aureas
```
91
What is Transient Synovitis?
```
2-12 yrs
common
often initiated by viral illness
may have fever and incr acute phase reactants
spontaneous resolution
```
92
Developmental hip dysplasia
at birth
'clicky hips' on Ortolani's and Barlow's manoeuvres
6-10/100 live births
93
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
```
94
What is Severe Combined Immune Deficiency?
usually X linked inherited
affects humoral and cellular components
-> early, severe, atypical infections
only possible cure - BM transplant
95
How many resp tract infections per year is normal
preschool age 6-8 is normal
| most will be self limiting viral infections
96
What causes secondary immune deficiency?
drugs
infection
malignancy
97
What are the causes of HIV in children?
vertical transmission:
in utero, during delivery, breast feeding
infected blood products
dirty needles
98
What are AIDS defining illnesses?
TB
| candidiasis
99
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
100
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)
101
What is the toxic dose of paracetamol?
>130mg/kg
102
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
103
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
104
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
105
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
```
106
Managing OD
admit all paediatric self harm / attempted suicide
107
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
108
Rosving's sign
Pain in RIF in response to left sided palpation
| Suggests peritoneal irritation in the RIF
109
McBurney's point
1/3 of distance between ASIS and umbilicus
| Typically tenderness and guarding is seen over this point in appendicitis
110
Cough sign
Pain in RIF after a voluntary cough
| Suggests peritoneal irritation in the RIF
111
Obturator sign
Pain on internal rotation of flexed right thigh
| Caused by inflammatory mass overlying the psoas muscle
112
MCADD
Medium Chain Acetyl co-a Dehydrogenase Deficiency
Inborn error of metabolism
Fat can't be broken down
Low energy + hypoglycaemic episodes
113
Ddx chronic cough
asthma, inhaled foreign body, sinusitis, post nasal drip, habit cough, allergic rhinitis, TB, CF, bronchiectasis, immune deficiency
114
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
115
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
116
Prehn's sign
Tenderness relieved by elevating the scrotum
| +ve indicates epididymitis
117
Bloom's syndrome
```
AR
Short stature
Facial photosensitive rash
Narrow face
Subfertility
Immune deficiency
```
118
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)
```
119
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
120
Barlow's test
Neonatal screening for DDH
Hip can be easily displaced posterior lay out of acetabulum
On adduction of leg with post pressure
121
Ortolani's test
Neonatal screening for DDH
Femoral head can be reduced back into acetabulum
On abduction of leg with anterior pressure
122
Hand foot syndrome?
Children with SCD
Present with tender swelling of hand/wrist/feet
Precipitated by stress or cold
123
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
