Paediatrics Flashcards
1
Q
What is the most common cause of epiglossitis
A
H.inflenzae type B
2
Q
What are teh acute asthma numbers
A
resp 30
HR 125
o2 92
3
Q
Asthma chronic pathways
A
SABA
iCS low dose
LABA
ICS medium dose
Add LTRA
Higj dose steroid
Add oral theophylline
4
Q
Well controlled asthma symptoms
A
exercise and sleep sumptomless
No cough or wheeze on most days
Inhaler used less than three time s a week
Normal lung function test
5
Q
Asthma crisis treatment
A
Oxygen
Salbutamol nebulised
Ipratroprium brimode nebulised
Oral pred
Iv hydrocortisone
Iv magnesium sulphate
Iv salbutamol
Iv aminophyllin
Intubate
6
Q
what are sighs oy an unwell child j resp.
A
raised resp
Use of accessory
subcostal recession ‘cyanosis ‘trachel tugging ‘head bobbing ‘nasal flaring ‘abnorla airway noises- wheezing, grunting stridor
7
Q
when to admit a child for bronchiolitis
A
*less than 3
Pre existing conditions - downs, premiturity, cystic fibrosis
* 50-75% less of their noraml milk intake
* Dehydratio
* Reap above 70
* Sats below 92%
* Moderate to severe rep disease
* Aponeas
Paretns unconfidant
8
Q
how long should chlren wrh scarlet fever take to returnt to school after startng antbotcs
A
24 hours
9
Q
what do xrays show for necrotsng enterocolts
A
dilated bowel loops (often asymmetrical in distribution)
bowel wall oedema
pneumatosis intestinalis (intramural gas)
portal venous gas
pneumoperitoneum resulting from perforation
air both inside and outside of the bowel wall (Rigler sign)
air outlining the falciform ligament (football sign)
10
Q
6 week baby check hip dislocation tests?
A
barlo - attempt to dislocate
orlani - relocate the hip
11
Q
what causes croup and whats teh treatment
A
paranflenza B vrus
stat dose of oral dexamethasone
12
Q
what would an ultrasoud of nturcussepton loo le
A
target sgn
13
Q
What are teh defects of tetralogy of fallout
A
PROV
pulmonaru stenosis
Rv hypertrophy
Overriding aorta
VSD
14
Q
what is teh criteria for infective endocarditis
A
BE TIMER
Bkood culture positive
Echocardiogram ecg evidence
Temp
Immunoloficak phenomena
Micro evidance
Embolic phenomenon
Risk factors
15
Q
What is teh investigation for puloric stenosis
A
ultrasound
16
Q
What s te treatment for patent ductus arterosus
A
idomenthacn
17
Q
what s the antbotc for whoopng cough
A
azthromycn
18
Q
What is kallmans syndorme
A
hypogonadotrophic hypogonadism and no smell
19
Q
what is kochers criteria
A
probability fo septic arthritis in children
non weight brearing
fever >38.5
WCC >12
ESR>40
20
Q
Where does neuroblastoma come from
A
neural crest cells in teh sympathetic chain for adrenal medulla
21
Q
What blood changes for tne ,ost common type ofnpaed blokd diswse
A
Acute lymphoblastic leuekemia
Pancytopenia
22
Q
what fracturer scake is used for pediatric
A
salter Harris
23
Q
Slipped femoral epiphysis definition and epidemiology
A
Head of femur is displaced form growth plate, boys 8-15 obese
24
Q
what s teh treatment of threadworms
A
mebendazole
25
6 weeks developmental milestones
smiles
26
4-6 months DMS
sits with support
reaches for objects
supports head
27
6-9 months
palmar grasp
curious and engaged with people
sts unsoported
turns head to name
28
12 months dms
cruising
pincer grasp
saying snlgle words
waves goodbye
claps
29
15 months DMS
tower of 2 bricks
walkng unaded
30
2 yeard DMS
run and kick ball
tower of brcs
31
3 year DMS
clmb tars one foot at a tmi
buld a brdge
basc sentances
32
pneumonia treatment
amoxclln or co amox
clarthromcni
33
cause of broncholosts
RSV
respratory syncytal cirus
34
whoopng cough cuase and treatment
bortadella pertussis
macrolides - azithromycin, erethromycin, clarithromycin
35
what causes cyanotic heart diseae
rght to left shunt
36
what are teh cyanotic heart diseases
truncus arterosus
transposton of teh great arterers
trcuspid atresa
tetralogy of fallot
asd
37
what s eisnmongrs syndrome
septal defect allows left to rght shunt cuasing pulmonary hypertention whch then leads to a rght to left shunt blood bypasses the lungs and causes cyanoss
cyanotic heart dsease!!!
38
causes of poor feedng
nfecton
teethng
premature - no such and swallow
CHD
down syndomre
39
tests and treatment for pylorc stenoss
* On examination after feeding, peristalsis will be visable in the stomach
* A firm roud mass can be flt that fells like a large olive
* Blood gas - hypochloric metabolic acidosis as they are vomiting up all the HCL
* Diagnose using an abdominal ultrasound to visualise the thickend pylorus.
* Laprospoic pyloromyootmy (ramstedts operation)
* This is an inscision to widen the canal.
40
# laxative types
treatment for consipation pharmacutical
movicol - stool softner
disimpaction regieme - stimulant laxative such as senna/sodum picobisulphate
41
what are teh 3 types of laxative
bulk - (fibre) fybogel (ispaghula husk)
osmotic - movicol, lactulose
stimulant - senna, sodium picosulfate
42
hirchsprugs symptoms, diagnosis and treatment
* Delay is passing myconium for 48hours
* Chroic consipation since birth
* Abdo aina dn distention
* Vomiitng
Poor weigth gain and falire to thrive
abdo xray
rectal bopsy showing abscence of ganglion cells
removel of aganglionic section of bowel
43
inturcusspeion key presentations
3 months to 3 years
*
* * Severe colicky abdominal pain
* Drawing their legs up
* Pale lethargic unwell child
* Recurrtnt jelly stool
* Right upper quadrant pass on palpation
* SAUSAGE SHAPPED
* Comiting
Intestinal obstrucntion
44
intercusseption test
ultrasound - target sign
contrast enema
45
intercusseption treatment
theraputic enema
46
cows milk protien allergy presentation and treatment
can pass through milk!!!!
abdo pain
vomiting
cough/sneeze
wartery eyes
eczema
urticarial rash
younger than 1 year
if breastfeeding avoid dairy products
hydroloysed formula, then climb teh milk ladder
47
mekles diverticulum test
technatioum 99 scan
ct
ultrasoud
48
key presintations of billary atresia and treatment
* Jaundice present shortly after birth lasing more than 14 days (21 in premmies)
* Pale stolls
* Dark urine
* 2-6 weeks old
Faliure to thrive
kasi poroenterostomy (attathcing a seciton of small intestine to the opening if the liver)
49
nephritic and most common cause in syndroem
nephritic - heamaturia, oliguria, mild proinuria
acute post strep glomerulonephritis
IgA nephropathy
50
nephrotic syndroem and most common cause
leaky BM in glomerulus casugin Oedema, protinuria and hypoalbuinaemia
minimal change disease
51
minimal change disease test snad treatment
* Renal biopsy and standard microscop wth show no abnormality
Urinanlysis will show small molecular wirght protiens and hyaline casts
Prednisolone - high dose initially
* Low salt diet
* Dieurtics for oedema
Albumin transfusions
52
acute post strep glomerulonephrtis pathophysiology, test and treatment
1-3 weeks after tonsillitis (strep pyonenes (A)). immune complexes get stuck in the glomeruli and cause inflamatio
thraot swab to test for tonsillitits
supportove, antihyoertensives
diuretics for oedema
53
IGa nepropathy path, key presintations
IgA deposits in the nephrons ofteh kidneys
* Purpura
* Joint pain
* Abdo pain
* Renal involvement
* They can develop arthritis, mainly I knees and ankles
* Can lead to GI haemorrhage and infarction
54
IgA nephrolagy tests and management
Biopsy -
“IgA deposits and glomerular mesangial proliferation
Supportive
Steroids sometimes
Cyclophosphamides
Close monitoring
55
test for vesicoureteric reflux
* Micuratin cystourethrogram - urinary cathaterisation and contract into eh bladder. Refluc is detected on voiding
Indirect cystogam radionucletide using scans a
56
path for heamolytic uraemic syndrome
* Antibiotics or antimotility drugs gven during the gastro enteritis make it worse!
* The formation of blood clots consumes plate,lets leading to thrombocytopenia, and the clots block u the kidneys
The clots also caus the heamolsis of oterh cells in small vessles as it causes tehm to rupture as ethy pass by
57
heamolytic uraemic syndrome key presentations
Classic triad f:
* Microangiopathic heamolytic anemia
* Acute kidney yinjury
* Thrombocytopenia
Previous gastroenteritis!!!
* Diahrrohea which turns bloody in 3 days
After 1 week the HUS symptoms develop:
* Fever
* Abdo pain
* Legarthy
* Pallor
* Reduced urine output
* Heamaturia
* Hypertention
* Bruising
* Jaundice
Confusion
58
heamolytic uraemic syndrome teartment
* Hosptial admission with supportive treatment of:
* Hypovolemia - (IV FLUId)
* Hypertension
* Severe anaemia - blood transfusion
Severe renal faliure - dialysis
59
what are 4 cuases of fevers for more than 5 days?!?!?/
kawasaki, stills, RF and leukemia!!
60
kawasaki disease diagnostic criteria
CRASH and BURN (4/5 mnor symptoms plus fever)
Conjunctvts
Rash
Adenopathy
Strawberry tounge
Hands and feet swelling
BURN (fever >5 days)
61
stepwise management fo croup
Oral dexamethasone
Oxygen
Nebulised budesonide
Nebulised adrenalin
Intubation and ventilation
62
presintatio of croup
Increased work of breathing
“Barking” cough, occurring in clusters of coughing episodes
Hoarse voice
Stridor
Low grade fever
63
age group of croup
6 months to 2 years
64
what conditions are tesed for on teh newbor screening rogram
cystic fibrosis (CF)
sickle cell disease (SCD)
congenital hypothyroidism (CHT)
phenylketonuria (PKU)
medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
maple syrup urine disease (MSUD)
isovaleric acidaemia (IVA)
glutaric aciduria type 1 (GA1)
homocystinuria (HCU)
65
what would the csf look lke in bacterial infection
Turbid
hight neutrophills
hgh protiens
high glucose
66
what does teh CSF look like in viral infection
clear
high lymphocytes
ormal protiens and glucose
67
what is teh management for sepses
blood clutures
urnie output
fluid
antibiotics
lactate
oxygen
68
what is stephens - jhonsosn syndrome and ts causes
a form of toxc epinermal necrolysis affecting less than 10% of eh body. it is the blisterinf and shedding of the top layer of skin from the body.
it is caused by antibiotis, antiepileptics, allopurinol, NSAIDs and infections such as HIV and herpes
69
what is the treatemtnet for stephens - jhonsosns syndrome
medical emergancy
steriods
immunoglobulin
70
what is a hemangioma
blood vessles forming a raised lump
appers soon after birth but diseaperds by age 7
71
what are teh causes of a non blanching rash
Meningiococcal septicaemia
HSD
leukeaemia
heamolytic uraemic syndrome
idiopathic thrombocytopenic purpura
72
scarlett fever cause, presentation and treatment ad isolation time
Strep a infection
sandpapaer rash on chest neck and arms , strawberry tounge
penV and go back to school in 24 hours
if no pen v, isolate for 2- 3 weeks
73
rosela infantum cause and presintation
snotty and unwell, got better and ONLY THEN developed the rash
starts on torso rhen streads to limbs
74
slapped cheek cause and presintation
parovirus B19
once uourve got the rash youre no longer infective
starts on face and mived onto torso and limbs
75
mealses key presintations
starts on fac eand soreads t rest of body
KOPLIC SPOTS - white spots in mouth!!
76
treatment for kawasaki
high dose asprin
IV immunoglobulins
Echocardiograms to look for cornary artery anyerisms
77
rubella preintatoin and managemtn
mild rash lasts 3 days
notafiable disease
children must stay ff school 5 dyas after rash
78
what is diptheria
toxin mediated bacteria caused by cornybacterium diptheriae
79
diptheria key presentations
* Sore throat
* Dysphagia
* Dysponea
* Croupy cough
* Swelling of neck
* Unvaccinated individual
* Fever
Stridor
80
treatment of diptheria
* Hospitallsation
* Potential intubation to prevent obstruction
* Cardiac monitering - myocarditis and HF can develop
* Droplet controll and isloation
* Dose of antitoxin!!!!
Antibiotic - procaine benzypenecillin or erythromycin
81
commonest causes of meningitis in kids and neonates
Children:
* Neissiera meningitidis
* Streptococcus pneumonia
Neonats:
* Group B strep from the birth canal
VIRUS:
* Herpes simplex
* Enterovirus
* Varicella zoster
- supportive treatment or aciclovir
82
meningitis key presintations in kids and babys
* Fever
* Neck stiffness
* Comiging
* Phptophbia
* Alerted consioiusness
* Seizures
Non blanching rash if septceamia.
Nepnetes and babys:
* Non specific
* Hypotonia
* Poor feeding
* Legarthy
* Hypothermia
Bulging fontanelle
83
when to lumbar puncture in meningitis suspect
umbar puncture in all cildren under 1 month with fever, 1-3 motnsh with fever and unwell under 1year wth fever and signs of seirous illness.
84
treatmentof meningitis
Community:
* If suspected meningitis and non blanching rash, Stat IM benzylpenecillin and imiadtiatl hosptial.
Hospital:
* Lumbar puncture.
* Bloods for meningococcal PCR
* Under 3 months – cefotaxime plus amoxicillin (the amoxicillin is to cover listeria contracted during pregnancy)
* Above 3 months – ceftriaxone
* Add vancomycin if there is risk fo penecillin resistant
* Steroids are used to redce neurologcal da,ge. - dexamethasone 4 times daily for 4 days if over 3 months old
*
85
public health side of minningitis
NOTAAFIABLE DISEASE
* People who have had contact 7 dayas before can hve a single dose oral ciprofloxacin given ASAP!!!
Close contacts - prolonged contact in household setting 7 days before onset of the illness or transient close contact but exposed to large droplets at hospital admissions
86
complications of meningitis
earing loss
* Seizures and epilepsy
* Cognitie imparement
* Memeory loss
Cerebral palsy
87
causes of encephalitis
* Most common is viral
* Herpes simplex 1 in children form cold sores
* Gerpes simplex 2 from genial herpes in neonates 9birth)
* Varicella zoster
Epstien barr
88
vaccination schedule to 1 year
8 weeks
* 6/1 -diptheria, tetanus, polio, H influenzea, hep B
* Menengiococcal B
* Rotavirus
12 weeks
* 6/1
* Pneumoccoal
* Rotavirus
16 weeks
* 6/1
* Meningiococcal B
1 year
* 2 in 1 HIB and Meningiococcal C
* Pneumococcla
* MMR
* Meningiococcal B
89
vaccination schedule 2-14 years
2-8 years
* Influenza virus
3 year 4 months
* 4/1 - diphtheria, tetanus, pertussis and polio
* MMR
12-13 years
HPV (6,1 - genitla warts 16,18 - cervical cancer )
14 years
* 3/1 - tetanus, diptheria and polio
* Meningiococcal A,C,W,Y
* whoopng cough
90
what are complications of not getting teh MMR is you cach teh diseases
* Measules: hospitlalization, death, blindness, pneumonia, seizures,
* Mumps: deafness, encephalitis, orchitis
* Rubella: deafness, cateractsm heart defects, brain sdisorders, sliver and spleen damage.
91
what is scalded skin syndrome and its presentation and management
Definition
Caused by staphylococcus aurus that produces epiermolytic toxins. Tese protease enzymes break down the protiens that hold the skin together and the skin breaks down
* Starts with genralised patches of erythema
* Looks thin and wrinkled
* Followed by the formation of flid and blisers called bullae leaving sore red skinbelow
* Looks lie a burn
* Nikolsky sign - getle rubbign of the skin causes it to peel away
Fever, legarthy, irratibilty, dehydration
Management and monitoring
* Admission and iV antibiotics
* Fluid and electolyte balances
* Ussually make fullr eceovery and don’t scar
92
severe combined immunodeficancy define and cause
The moost severe immunodeficancy disease hwhc is a syndreom caused by genetic disorders resultin in absetn or dysfunctioning T cells
* Over 50% caused by mutation in common gamma chain on the x chromosne
* X linked recessive inheritance
93
severe combined immuno deficancy presentations and treatment
N the fisrt few months of life -
* Persisatant sevre diarrhoea
* Falire to thrive
* Oportunistic infections
* Unwell after live vaccines - BCG, MMR, nasal flu
* Omenn syndrome - a rare cause of SCID, autosomal recessive and results in mutation of RAG 1/2. the abnormal T cells attack the foetus causing rash, hair loss, diarrhoea lymphadenopathy, hepatosplenomegaly.
* Speclist cenre
* Imunoglbulin therapy
* Sterile enviroment
* Avoid live vaccines
* Hematopoietic stem cell transplant.
94
reye syndrome define, causes and presentations and treatment
reye of sunSHINE
steatoss
hepatomegally
nfecton (vsV)
not awae
#encephalopahty
*
* * Acute encephalopathy with hepatic dysfunction stemming from mitochondrial damage.
* Virla infection
* Exogenous toxins
* Drugs
* ASPRIN
* Vomiting
* Risk factors - ercent viral infection
* Altered mental state
* Hyperventilation
* Hepatomegally
* Diminished pain respinse
* Seizures
Management and monitoring
* Supportive care
* ABCDE
* Vital dign and urine moniyering
* Look for signs of raised ICP
95
what are febreil convulsions - everythign
Sezures in childrenn aged 6 months to 5 years who also have a high fever.
* Simple - pmly occur once, last less than 15 mins and are genralised tonic clonic
* Complex- partial or focal seizure, lst more than 15 mins ot occur multiple times
All other neuology ,must be ruled out:
Epilepsy, meningitis, heamoegae and tumor, syncopeal episodes, electoltye sbnormalaties, trauama,
Simple just treat the underlying cuases.
* Stay with child, put them on a capted floor, recovery position, call an ambulance if it lasts more then 5 mins
Not ususally any long lasting damafe
Can increase chances of developing epilepsy
96
define and manage tonic clonic seizures and focal seizures
Genralised tonic clonic:
* Muscle tensing and jerking
* Tonic (clending) comes first
* Tounge birint, incontinance and irrecgualr breathing
* There is a post ictal period where the person is confused irrabtble drowsy or low
Manage with sodium valporate
Second line - lamotrigine or carbamzapine
Focal
* Start at the temporal lobe
* Affect hearing speech, memoery and emotions
* Halluciations, memory flashbacks, déjà vu, straneg things on autopilot
* Carbamazaptine ot lamotrigine
* Socium valporate or levetiracetam
97
define and manage abscene (and test) and atonic seizures
Absence
* Trypical in children
* Pateint becomes blanck, stare into s[ace and abruptly returns to normals
* To induce the seizue ask the patiet to hyoerventilate (bkow a pin wheel)
* Last 10-20 seconds
* Sodium valporate, ethosuximide
Atonic
* Drop attacks
* Brief lapses in muscle tone that last less than 3 mins
* May be ndcatve of lennox gastut syndrome
* Saodum valporate lamotrgne if it doesn’t work
98
define and manage mycclonic and inflantile spasms
Myoclonic
* Breif suffen contractions
* Sodium valporate
* Other: lamotrigine, topirmate
Infantile spasms
* Also called west syndreom
* Starts at infancy is is ull body spasms
* Prednisoloe
99
what are teh side effects fo sodium valporae, carmbamazapine and phenytoin
* Teratogenic
* Hair loss
* Tremor
* Liver damage
Agranulocytossi
Anaplasitc anaemia
Induces P450
* Folate and vitmain D deficiancy
* Megaloblasitc anaemia
* Osteomalacia
100
status epilepticus define and treament
Status epilepticus:
* Lasting more than 5 mins or more than 2 without regaining consciousness
* Secure the airway
* High conc oxygen
* Chec blood clugose
* Gain IV acesss
* IV lorazapam, - repeat after 10 mins
* OV phenobarbital or pheytoin or levaracitam
Community emergency meds
* Buccal midazolam
Rectal diazapam
101
what s teh treatment for hypothyrodsm
levothyroxne
102
what is hypergonadotrophc hypogonassm and some cuases of t
No negative feedback fomr the sex hormones leading to high gonadotrophins and low sex hormones.
* Precious gonad damage - testicual torsion, cnacer, infections (mumps)
* Congnital absence
* Kleinfelters syndrome (XXY)
Turners syndrome (XO)
103
what s hypogonadotrophc hypogonadsm and some causes for t
* Deficinacy of LH and FSH
* Abdnomalt functiong of the hypothalamous pr pituitry
* Damage - radiotheray or surgery #
* Hrowth horme deficancy
* ##hyperprolactineamia
* serious chronic conditions - cystic fibrosis or IBD
* Excessive exercise r dieiting
Kallmans syndrome - genetic condition causing hypogonasdotrophic hypogonadism. Associated with reduced or absent sense of smell
104
what s congental adrenal hyperplassa
Congenital defiancy of the 21-hydroxylase enzyme which causes unerproduction of cortisol and aldosterone and overproduction of androgens from birth.
Autosomal recessive patterns.
105
what s teh classc sever and less severe presentaton of CAH
SEVERE CASES:
Shortly after birgh:
* Hyponatermia, hyperkalemia and hypoglycemia
* This leads to poor feeding
* Vomiting
* Dehydration
* Arrhythmias!
* in females - virulised genetalia (ambigious) due to the high testosterone levsls
LESS SEVERE:
Females:
* Tall for age
* Facial hair
* Absent periods
* Deep voice
* Early pbity
Male:
* Tall
* Deep voicce
* Large pens
* Small testicles
* Early pibity
HYPERPIGMENTATION!!! - this happens because the anterior pituitry produces lots of ACTH in response ot the low cortidol levels!!
* Classc prentaton n boys - salt wasting crisis a few weeks after birth
Classc presintationin girls - undfferentaled genatala at brth
106
what s teh treatment of CAH
* Hydrocortisone
* Aldosterone replacement - flucortisone
Female atients may require surgery
107
defne androgen nsensvty and ey presentatons
x lned recessve condtons where cells are unabel tot react to androgens
Genetically male with XY characteristics
* Femal phenitype
* Thery have tested in the abdomen or inguinal canal that nerver descnded
* Absence of a uters upper vagina, cervicm fallopina tubes and ovaries
* The internal female organs don’t develop because the teste produce anti-mullarina hprmane whch preventsteh formation of the upper vagina, cervi and fallopian tubes
* Taller than average
* Often present with inguinal hernias containg testes at birth
* Or primay ameh=nhorea at puberty
* Rasied LH
* Normal or raised FSH
* Notmal or raised testosterone for a mle
* Raised oestrogen for a male
108
presntaton of dabetc etoacdoss
Polyuria
Polydipsia
N&V
Acetone smell on breath
Refuced GCS
Symproms of underlying trigger - sepsis
109
treatment of DKA
* Correct dehydration over 48 hours
* Give a fixed rate of insulin, use https://dka-calculator.co.uk/
* Add potasisum to the saline soloution!!!
* Use iv dextrose to help controll the glucose levels
Mobiter glucose, ketones, pH and
110
pathology of scel cell anaema
Heamolytic anamia. The pateints have HbS, which is abnormal sickle cells. It is autosomal recessive where the beta globin gene on chromosone 11 results in the sickle cell trait.
Having one of the genes is beneficial against malaria. Having 2 is bad and leads to sickle cell disease.
111
key presentaton of sckle cell anaema
* Anaemia
* Increased risk of infection
* CKD
* Sickle cell crisis
* Stroke
* Avascualr necrosis ]
* Gallstones
* Prapism - painfu and persistant erections
Sickle cell crisis:
* Acute exastubation
* Spontaoursy triggerd ot by dehydration, infection, stress, cold weather
* Vasul-occulative crissis - the most common type, sickle red blood cells block peripheral capilleries causing ischeamia
* Pain and swellig of hands and feet. Fever
* Splenic sequestration - blocked blood flow to the spllen leadint o anaemia and shock
* Emergacy - need blood tranfusion and fluid rescusitation
* Splenic infarction ca occur - hyposplensm leadint o suseptability to infection - particularly to strp pneumonia and heaophill influenzea
* Splenecomty
* Aplastic crisis - tempoary absence of creatin new blood bceks after parovirus B19 leads to anamina needsing blood transfusion normall lasts a week
* Acute chest syndrome - vessles in lings become cloged. Triggerd by occulsive crisis, fat embolism ot an infection
* Medical emergancy with high mortality
* Analesisia
* iV fluids
Antibiotcs blood rasfusion, oxygen
112
anagement of scle cell anaema
ickle cell crissis:
* Manage supportivly
* Treat infections
* Keep warm
* Good hydration
* Analgesia
* Low threshhld for hospital admission
General manegement:
* Avodi triggers
* Up to date vaccines
* Antibiotics prophylaxis - pen V
* Hydroxycarbamide - promotes HbF
* Crinanlizumab - monoclonal antibody targeting p-selction, an adhesion molecule in endothelial cells and platelts. It prevent red blood cells from sticking and casuign a vaso-occlusive crisis.
* Blood transfusion for severe anaemia
Bone marrow transplant
113
what s facon anaema
Definition
Rare autosomal recessive condition leading to progressive bone marrow failure affecting all 3 haematopoietic cell precursors.
114
ITP management and complkicatios
* Monitered untill plateltes return to normal
* Prednisolone
* IV immunoglobulins
* Blood transfusions #
* Platelet transfusions
* Most wll self resolve after 3 montsh
* Chrnoci ITP
* Anaema
* Brain heammorage
* GI bleeding
115
defne and presentatons of ITP
Purpuric rash caused by t2hr causing the destructioni of platelets.
Spontenous or trggered by viral infecton
* Recent viral illness 24-48 ours ago
* Bleeding
* Bruising
* Petechia or purpuric rash
116
5 thngs taht ncrease rs of leuaema
* Radioation exposre - abdo xray during pregnancy is the main enviromenta risk factor
* Down syndreom
* Kleinfelters sunfreom
* Noonan syndreom
Faconi anaemia
117
presentatons of leukaemia
It causes pancytopenia - causing anaemia, leukopenia, thrombocytopenia.
* Fatigue
* Fever
* Falirue to thrive
* Weight loss
* Night sweats
* Pallor
* Pethecia (thrombocytopenia)
* Abdo pain
* Lymphadenopathy
* Persistant bone pain
* Hepatosplenomegally
118
tests and treatment for leukemia
* Refer any children with perechia or hepatomegally
* Urgery full blood cout withtin 48 hours
* FBC showing anaeia, leukopenia, thombocytope aand high numbers of abnormla WBCs
* Blood film - blast cells
* Bonoe marrow biopsy
* Lmoh node biopsy
* Chest x ray
* T scan
* Lumbar puncurre
* Umunophenotyping of the abnormal cells
* Chemo
* Radiotherapy
* Bone marrow transplant
Complcations f chemo:
* Flaiure to work
* Stunted growth
* Neurotoxicity
* Infertilty
* Secodary malignancy
* Cardiotoxicity
119
wilms tumour all
* Mass in abdomen
* Abdo pain
* Hematuria
* Legarthy
* Fever
* Hypertention
* Weight loss
* Ultrasoud
* CT or MRI to stage eth tuour
* Biopsy and histology to make a complete diagnosis
Surgical excisions
Chemotherapy and radiotherapy
120
symotoms of neuroblastoma
* Non specific and variable
* Palpable mass
* Compression of nerves - can causes urinary incontinance
* Bone - pain or limp
* Lymphadenopathy
* And pancytopenia
* Sweating, pallor, watery diarrhoea, hypertension
* Hypertention and tahcy due t excessive calocamine production
* Scatterd purpura - blueberry muffin rash!
Thrombocytopenic prupura
121
test and management of neuroblastoma
* Urien catchelocamine to creatien ration
* The key is to look for the products of carchelochamine break down - raised HVA/VMA in urien.
* Hva - homovanillic acid
* VMA - vanillymandelic acid. 90% of patient will ave these raised!
* Ultrasoudn, MRI
* MIBG scan - radioactive isotope and 2 scans are taken 24 hours apart.
* Can regress in youger children
* In older children, treat moe agressivly.
* Surgery
* Chemo and radiotherapy
* Immunotherapy
* Antiemetics and parinkillers
122
concetrnng sgns of headaches
* Constant headache
* Nocturnal
* Worse on waking
* Wore on coughing straining and bendng forewards
Vomtng
123
ey presentatons of bone tumoru
* Persistent bone pain - worse at night
* Bone swelling
* Palpable mass
Restricted joint movements
124
what s ewngs sarcoma
* Tumour occuring in bone and soft tissue
* Ocalised pain and welling with pathological fracture
* More common in daiphysis han metaphysis
* Commoy metastasies to lung and bone #
* Xrya , biopsy, lactate dehydrogenase
* MRI CT isotope bone scan
* Chemotherapy, surgery chemo
125
what s teh blood marer for lver touur
AFP - alpha feto proten
126
defne and ey presentatons of klenfelters
Males have an additional X chromonsone making them XXY.
* Part way though pubty they develp hypergonadotrophic hypogonadism
* Often undiagnosed untill investigation for infertitlity
Appear as nomal males unitll pubity
* Taller heigt
* Wider hips
* Gyneacomastia
* Weaker muscles
* Small tsticles
* Reduced libido
* Shyness
* Injertility
* Subel learing difflcutie - speech and language
127
management and complications of klienfelters
* Testosterone injectiosn
* Advanced IVF tecnuiqes
* Breast reduction
* Speech and lanuae theray
* Occupational therapy
* Physiothera
* Educational suppoort
Slightl increased risk of:
* Breast cancer
* Diabeteris
* Osteoprossis
* Depressiona nd anxiety
128
defne and ey presentatons of turners syndrome and amanagement
* Short stature
* Webbed neck
* Broad chest with wirley spaced nipples
* High arcing palate
* Downward slopng eyes and ptosis
* Underdeveloed ovares
* Late pubity
* Infertility
cubtal valgus
XO chormonsome
treat eth hrowth hormone therpay
129
ey presntatons of downs syndorme
* Hypotonia
* Brachychepaly
* Short neck
* Short stature
* Flattened face and nose
* Prominent epicanthic folds - medical eye and eyelid slin
* Upward sloping palpebral fissures - gap bewtween lower and upper eyelid
Single palmar crease
130
complications of down synroem
* Learning difficulties
* Recurretn otitis media
* Deafness and eustachian tube abnormalaties
* Visual problems such as myopia, strabimus and cateracts
* Hypothyroidms
* Cardiac defects
* Atlantoaxial instability
* Leukaemia
Dementia
131
what is pataue syndreom
trsomy 13, not compoatible wit life
132
what s edwards syndreo
trisomy18
* Rocckerbottom feet
* Dysmorphic features
Learning disability
133
what is achondroplasia -
* The gene fibroblast growth factor receptr 3 on chromosone 4
* It an happen from sporadic mutation or the inheritance of an abnormla copy of a gene
* Autosoma domminant patter, homyzgenous gene muation is fatal in the neonatal period, therefore tey must have one copy of a normla gene
It causes abnormla fnction in the epiphysal growth plates
134
key presentaiot and compliations of achondroplasia
* Disproportionate short stature
* Average height of 4 feet
* Femur and humers more affected than forearm and lower leg
* Tunk length and intelligence are average
* Short digits
* Bow legs
* Disproportionate skull
* Foramen magum stenosis
* The skull base grows and fuses weirdly leadin to a flattentd midface and nasa bridge and the cranial vaul grows and fuses via membranous ossification
* Recurrent otitis media
* Kyphoscoliosis
* Spinal stenosis
* Obstructive sleep apnoea
* Obesity
Cervival cord compression and hydrocephalus
135
key presentationi of angelmans
* Delayed development
* Server delay or absence of speech
* Ataxa
* Fascnaton with water
* Happy demeanour
* inaproprate laughter
* Hand flapping
* Abnormal sleep patterns
* Epilepsy
* ADHD
* Dysmorphic features
* Fair skin, light hair, blue eyes
Wide mouth and widely spaced teeth
136
key presentation fo prader wlls
* Constant hunger
* Obesty
* Hypotoa
* Mld -moderate learnng dffculty
* Hypogonadsm
* Dar soft sn prone t brusng
* Anxety
* Dysmorphic featres
* Narrow forehead
* Almond eyes
* Stabsmus
* Thn upper lp
Downturned mouth
137
ey presentaton of noonans
* Short stature
* Broad forehead
* Downward slopng eyes
* Ptosis
* Hypertelorism - wide space between eey
* Prominent nasolabial folds
* Low set ears
* Webbed neck
* Widely spaced nipples
Associated conditions:
* CHD - pulmonary stenosis hypertrophic cardiomyopathy, ASD
* Cryptorchidism - leading to infertility in men
* Bleeding disorders
* Lymphoedema
Increased risk of leukaemia and neuroblastoma
138
key [resentaiton of willams
* Broad forehead
* Starburst eyes
* Flat nasal bridge
* Long philitrum
* Wide mouth and widely spaced dteeth
* Big smile
* Small cin
* Very socialble and trusting
Mild leadning disabilty
139
key presentatons of osteogeness mperfecta
* Recurrent and inapropriate fractures
* Hyperboblity
* Blue/grey sclerea
* Triangular face
* Short stature
* Deafnessf rom esarly adulthood
* Dental issues
* Bonde deformityr - bowed legs
Joint and bone pain
140
ey presentatons of rickets
* Legarthy
* Bone pain
* Swollen wrists
* Bne deformity
* Poor growth
* Dental problems
* Muscle weakness
* Abnormal fratue
* Bowing of the egs
* Knowkc kness
* Rachitic rosar - lums alog the chest
* Craniotabes - soft skull
Delayed teeth
141
what s transent synovtis
rratible hip caused by transier iritation of the synovial membrain n the joint. Most comon cause of hip pain in kids 3-10. associated woth recent upper resp viral infection.
142
key presentaton of trasent synovitis
* NO FEVER!
* Few weeks after viral illness
* Limp
* Refusal to weight bear
* Groin or hip pain
* Mild low grade temp
Otherwise well
143
management of transent snovts
* Analgesia
* Exclude septic artheritis!
* Clear safety net advice to attend A&E if they develp a fever or it gets worse
Followed up at 48 hours and 1 week
144
septc arthrts bactera
* Staphylococcus aureus
* Neissiera gonnorhoea
* Group a strep
* H influenzie
E coli
145
ey presentaton of septc arthrts
* Hot, red, swollen painful joint
* Refusing to weight bear
* Stiffness and reduced range of motion
* Fever
* Legarthy
* Sepsis
Affects a single joint - knee or hip
146
what is perthers disaease
Disruption of bloodflow to the femoral head leadig t avascualr nerosis. It affects the epiphysis which affects bone distal to the growth plate.
* Ages 4-12 most commonly 5-8.
* Boys
* Idiopathic
147
key presentatio sna tests for perthes
* Pain in hip or groin
* Limp
* Resticted movement
* Reffereed pain to the knee
* No history of trauma
* Xray
* Blood tests - normal
* Technatioum bone scan
* MRI scan
148
treatmetn for perthers disease
* Severity varies
* Conservatve to antain a helathy position and alingent of the joint
* Bed rest
* Traction
* Crutches
* Analgesia
* Physiotherapy
* Regular xrays to asses healing #
Surgery
149
slpped femoral epiphysis define, risk factors, key presentations
The head of the femur is displaced form the grwoth plate.
Boys
Age 8-15 but average age of 12
Obesety
* Adolescent obese boy undergoing a growth spurt
* History of minor trauma but pain is disproporitaite
* Hip, groin, thigh and knee pain
* Resticted range of hip movement
* Painful limp
* Resticted movement in the hip
* They will have restricted internal rotation
## Footnote
i am very bored
150
define and key presentatons of osgood schlatters
Inflamation AT THE TIBIELA TUBEROSITY WHERE THE PATELLA LIGAMENT INSERTS.
Common cause of anterior knee pain
10-15
Boys
Usually unilateral
* Stress from rubbing jumpin at the same time as the epiphyseal plare results in inflamation. This cause small avulaion fractire where the ligaent pulls away tiny pieces of bone
* This leads to growth of the tibieal tuberoscity causing a visavble bump below the knee
* Hard lump below knee
* Pain at anterior knee
* Pain exascubate dby physical activity, kneeling and extention
151
developmental displasia of teh hip 3 ris factors
Epidemiology and risk factors
* First degree FH
* Breech from 36 weeks
* Multiple pregnancy
152
developmental displasia of the hippresentaton, test and management
* Different leg lengths
* Restricted hip abduction on one side
* Significant bilateral restriction in abduction
* Difference in knee level when flexes
* Clunking on special tests
* Ortolanii test - knees and hips flexes, abduct the hp
* Barlow test - flexed hips and kness, hips adducted
* Clicking is common, clunking is not and requires ultrasound
* Ultrasound
* Xray
* Pavlik harness if under age of 6 months
*
* It keeps the hips stabe and alowsth eacetabulum to devleip to a normal shape
* Surgery if over 6 months, a hip spica cast immobilises the hip afterwards
153
what are the 5 types of JIP
* Systemic
* polyarticualr
* Oligoarticuar
* Enthetisis related
Juvanile psoriatic
154
systemeic JIP
Systemic
* Also known as stills disease
* Subtle salmon pink rash
* High fevers, lymphadenopathy , weiht loss
* Joint inflamation and pain
* Splenomegaly
* Muscle pain
* ANA antibodies nd RF negative
* Raised ESR, CRP, platelets and serum ferratin
* Key complication of macrophage activation system, where there is DIC< anaemia, thrombocytopenia, bleeding and it is life trheating. There is a low ESR!!!
155
polyarticular JIP
Polyarticular
* Involves 5 or more joints
* Symetrical affectign small joints and hands and feet, also hips and knees
* Mild fever, anaemia reduced growt
* The equiverlent of FA in adults
* RF negative noramll and
156
oligoarticular JIP
Oligoarticualr
* 4 or less joints
* Knee or ankle
* Common in girls under 6
* Anterior uvitis is common
* No systemic symptoms
* Inflamatry marers normal
* Ana antibodies posative, rf ne
157
enthesis related JIP
Ethesitis related arthritis
* Males over 6
* Pads seronagatve spondyloarthropathy
* HLA B27 positive
* Look for psoriasis and IBD
* Prone to anterior uveitis
* Prone to enthersis (inflamation at tendon atatchement sites)
* Wrist, interphalaneal joints in hands, wrist, greater trochaanter of hip, base of achillies, patella tendon,
158
junaile related arthertitis
Juvanile psoriatic artheritis
* Symetrical polyarthritis afecting smal joints, or asymetrical large joints
* PSORISIS
* Placques on skin
* Nail pitting
* Onychylosis
* Dactylisis
* Enthesis
159
treatments for JIP
* Exercise
* Phsychological support
* NSAIDs
* Steroids
* DMARDS - methotrexte and sulfasalazine
* Biloogical therapy - u=tumour necrosis factor inhiibitors sucha as adalinimab
* Splints to prevent deformity
* Eye screening for uvitits
160
what is torticollis
* Within the fist few weeks of life a small swelling is noted on the bays neck. It is a small fibrosisin eth sternomastoid muscle.
* Shortening of the muscle leads to troticollis (neck muscles spasaming and moving to one side)
* Shrtening of the SM results in the roation and tilting of the head ot the oposite side
* Passive excerciseded to allow the neck to achieve full movements
* Hemifacial atrophy and strabismus may develop unless the fell movement is restored
Physiotherapy!
161
what s the apgar score
appearance pulse grmace actvty respraton
all scored out of 2
162
newborn lfe supprt
delay cord clampng
dry/ stmulte/ warm up
5 inflaton n air then PEEP
apply O2
sucton laryngeal mask and consider inflation breaths
synchronise 3 chest comprssion to 1 ventilation
consider intubation
163
what s respratory distress syndreom
premmies who arent producing wnough surfactant - before 32 weeks
164
management and complication of respiratory distress syndrome
antenatal sterods given to mother
intubation and ventialtion
CPAPA
supplementrry oxygen
pneumothrooorax
infection
aponea
intraventricular heamorahe
necrotising enterocolitis
165
bronchopulmaonry dysplasia - define, presenration and prevention
chornic lung disase of prematurity
affects babies born befroe 28 weeks, fragile still lungs that arent full ydeveloped
guve dexamethasone antenatally, use CPAP rather than ntubaton
use caffne to stmulate rep effort
166
meconum aspraton - defne path presentatons
perstalss due to feotal dstress leadng to partal or total obstructon of teh ar. ths causes feotal hypoxa due to VQ msmathc
ncrease n pulmar ressur eleads to rght to left shuntning
it leads to inhibition of surfactant and causes inflamtion due to teh chemical in there
resp distress soon after birth
167
manageme t of feotal meconum nspraton
extrdte mecomiu to prevent further hypoxia
supplamental O2
surfactant
antibiotics
ECMO
168
what s ECMO
Extracorporeal membrane oxygenation (ECMO) is a treatment that uses a pump to circulate blood through an artificial lung back into the bloodstream of a very ill baby.
169
hypoxc shaema encephalopathy path and causes
hypoxa durng btg hleadng to permenrant damage and cerbeaal palsy
causes - maternal shock, intrapartum heammorage, prolapsed cors, nuchal cord
170
what are teh stages of hypoxic ischaemic encephalopathy
mild - poor feeding, resloves in 24 hours
moderat - poor feeng letharc hypotona sezures 40% develop cerebal palsy
sevre - reduced consiousness, 50% nortality, 90% decelop cerebal palsy
171
treatment of hypoxic ischeamic encephalopathy
supportive care and rescusitation
acid base baance, treatment of seizures
theraputic hypothermia is used in certain situation to protect the nrain. call to 33-34for 72 hours and thn warm up a=over 6 hours
172
what are the torch nfectons and bow are they transmiited
toxoplasma gondii
other agents - prepeenama pallidum, varciella zoester, parovirs B19, HIV
rubella
cytomegalovirus
herpes simplx virus
mum to babay - in utero, though teh birth canal or through breast milk
173
ey opresintation so ftorch infections
* Fever
* Legarthy
* Cateracts
* Jaundic e
* Rebrown spots on skin
* Hepatosplenomegally
* CHD
* Microcephallly
* Low birth weight
* Hearing loss
* Blueberry muffin rash
* Vision loss
* Deafness
* Intellectual disability
Seizures
174
what is necrotising enterocollitis
disorder afectign premmies when part o teh bowel becomes nectoric - leads to perforation, peritonitis, shock and death
175
key presintation of necrotising enterocolitis
intolerance to feeds, vomiting green bil, genrally unwell, distended tender abdomen, absent bowel souds, blood in stool
176
tests for necrotsnf enterocolts
FBC CPR capllery bloog gass
abdo xray -
Xrya:
* dilated bowel loops (often asymmetrical in distribution)
* bowel wall oedema
* pneumatosis intestinalis (intramural gas)
* portal venous gas
* pneumoperitoneum resulting from perforation
* air both inside and outside of the bowel wall (Rigler sign)
air outlining the falciform ligament (football sign)
177
mangment adn complications of necrotising enterocolitis
* Nil by mouth
* IV fluids total parentla nutrition
* Antibiotcs
* NG tube
* Surgical emergancy
Temporary stoma
perforation sepsis death strictures abcess
178
neonatal jaundice normal and abnormla cause
normla - mild janidce 2-7 days of age due to HgF breakdown. resolved by day 10
nicreased billirubin production -
heamolytic disease of teh newborn
ABO incompatailoity
heamorrag e
intraventricular heamoraage
polycythemia
sepsis and DIC
GP6D deficaincy
premature clearance -
prematurityy
breast mil
neonatal cholestasis
extrahelapci bilary atresia
hypopituitry
gilbrets syndorme
179
what is teh definitoi of prolonged neonateal jaundice
longer tahn 14 days in term and more than 21 in premmies
look for billary atresia, hypothyroidism and G6PD deficiancy
180
tests to doin neonatal juandice when prolonhed
* FBC and blood film
* Conjugated billirubin levels - hepatobillary cause
* Blood type testing
* Direct cooms test for heamolysis
* Thyroid function
* Blood and urine cultres
Glucose phosphare dehydrogenase levels
181
treatment for neonatal juandice
* Total millirubin levels can be monitered on treatment threshold charts
* T is counted in ours since birth
* Phototherapy - converts unconjugated billirubin into isomer so it an be excreted in the liver
* Eye patcehes and blue light is used form a ligh box
Exchange transfusion
182
kernicterus definition
Kernicterus:
* Brain damage to the bilirubin levels
* Bilirubin can cross the blood brain barrier and causes damage to the CNS
* Bulds up n the basal gangla and cerabellum
* Floppy, drowsy baby with poor feeding
* They wll get cerebral palsy, learning disability and deafness.
183
what s gastroshss
full thickness abdomnal wal defect where teh prgans protude outsde wth no prtectve membrane cuasng exposure to amntotc fludwhch elads to nflamaton
184
rs factros for gastroshsis
maternal smong
under 20
envromental expose
use of asprin and ibruprofen
185
treatment for gastroshisis
* Early detetion through scas
* Women induced at 37 weeks
* Immidiate fuid rescusitation and maintain adequqte temprature
* Sterile clean covering over the herniated contect to prevetn exaporation, heat loss and infection
* Surgery is defeinitive
Need a nasogastric tube and parenteral feeding whilst the inflamatory peel recovers
186
bowel atresa key presntations
bile stained vomit
subbtle bubble sign gas in stomach
187
signs of maternal hyperthyroidism in a neonate
small size
preterm
goitre
tachycadria
hepatosplenomeggaly
microcephally
188
bactera for neonatal sepsis
groiuo b streo
eli
listeria
klebsitelka
staph aureus
189
symotoms of neonatal spesis
fever
resuced tone
resp distress
HR abnormalityd
siezures
vomiting
juandice in teh firt 24 ohrus of life
hypoglycaemia
sepsis in mother
signs of shock
signs of shock
190
management of sepsis in a neonate
septic screen -
benzypenicillin and gentamycin
check crp after 24 hours
crp
191
what are teh catogries of teh peads traffic light system
colour
respiratory
circulation and hydratio
other
192
a,ber signs
pallo r
not responding normally
nasal flaring, >40 RR
<95% resp sats
tacy cardai, reduced mucous, poor feeding
temp >39
fever>5 days
swelling of limb
non weifht bearing limp
193
red signs
pale/blue
no response to cues, asleep
grunting, tachyopena .60
reduced skin turgour
<3 months temp >38
bluging fontnelle
non blanching rash
focl seizures
status epilepticsu
neck stiffness
194
fragile X syndrome signs and protien
fragile X r=mentl retardation protien (FMR1 gene)
LARGE EARS
long face
high forehead
lareg testes
hypermobile joints
low IQ
autism
195
196
what s teh crtera called for rhematc fever and nfectve endocardts
rf - jones
E - dukes
197
key presentations of RF
cardomegally
artheritis
subcutanous nodules
erythema marginatm
sidenhams chorea
198
what does an ASD murmur soud like
crechendo decrechendo murmur with fixed splid second heart soud
loudest ar upper left steral baorder
199
treatment for ASD
transvenous catheter closre or open heart surgery
anticoagulents can be used to reduced chances of clots and stroe in adults
200
what genetic conditions aare associated with VSD
dowsn syndreom
turners syndrome
201
murmur heard with a VSD
pan systolic hear loudest left lower sternal baorder
other causes of pansystolic - mitral and triscuspid regurgitation
202
what isteh creiteria used for IBS
rome 2
203
causes of appendicitis
feacolish
filieal worms
undigested nuts and seeds
204
further nvestgatons for UT
< 6 montsh need ultrasoud in 6 wees
reurrent UTI need ultrasoud n 6 weeks
atypcal UT need uptrasoud n llness
205
206
207
developmental mlestned form 3-12 months
3 months Little or no head lag on being pulled to sit
Lying on abdomen, good head control
Held sitting, lumbar curve
6 months Lying on abdomen, arms extended
Lying on back, lifts and grasps feet
Pulls self to sitting
Held sitting, back straight
Rolls front to back
7-8 months Sits without support (Refer at 12 months)
9 months Pulls to standing
Crawls
12 months Cruises
Walks with one hand held
208
developmental mlestones 13 months to 4 years
13-15 months Walks unsupported (Refer at 18 months)
18 months Squats to pick up a toy
2 years Runs
Walks upstairs and downstairs holding on to rail
3 years Rides a tricycle using pedals
Walks up stairs without holding on to rail
4 years Hops on one leg
209
