paeds take 2 Flashcards

1
Q

Is bronchiolitis an upper or lower RTI?

A

Lower

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2
Q

Most common viral cause of bronchiolitis

A

RSV

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3
Q

Age group affected by bronchiolitis

A

Age 1-9 months

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4
Q

RF for bronchiolitis

A
  • Brest fed <2 months
  • Smoke exposure
  • Siblings
  • Chronic lung disease due to prematurity
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5
Q

Bronchiolitis pathophysiology

A
  • Proliferation of goblet cells –> excess mucus production
  • Inflammation, bronchiolar constriction
  • Lymphicytes –> submucosal oedema
  • Mucus, oedema and increased cells in bronchioles –> hyperinflation, increased airway resistance, atelectasis and VP mismatch
    bronchioles inaflame, produce mucus, oedema and necrosis cause obstruction
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6
Q

S+S of bronchiolitis

A
  • Coryzal Sx precede cough and breathlessness
  • Dry wheezy cough
  • Cyanosis
  • Tachypnoe and tachycardia
  • Recession
  • Hyperinflation
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7
Q

Ix bronchiolitis

A
  • Swabs
  • Urine and blood if pyrexic
  • Examination
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8
Q

When to admit with bronchiolitis

A
  • Apnoea
  • <92%
  • <70% normal drinking volume
  • Severe resp distress
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9
Q

Bronchiolitis Mx

A
  • Supportive
  • Oxygen
  • Fluids if dehydrated
  • CPAP if struggling to breathe
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10
Q

Croup

A

Common viral childhood illness

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11
Q

Age of croup

A

6 months to 6 years

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12
Q

Most common croup organism

A

Parainfluenza virus

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13
Q

S+S croup

A
  • Few days onset
  • Coryza preceding
  • Severe barking cough
  • Harsh rasping stridor
  • Worse at night
  • Temperature
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14
Q

Ix croup

A

CLinical diagnosis

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15
Q

Mx croup

A
  • All children with mild, moderate or severe - single dose or oral dexamethasome
  • Can be managed at home if mild
  • Severe upper airway obstruction = nebulised epinephrine with oxygen facemask
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16
Q

Neonates pneumonia organisms

A

GBS
E coli
Klebsiella
S aureus

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17
Q

Infants pneumonia organisms

A

S pneumoniae

Chlamydia

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18
Q

whooping cough mx

A
  • exclude 48hrs from abx or 21d onset
  • <1m old = clarythromycin 1st pr axith
  • 1-12m = azith and clarith
  • > 1y = azith and clarith
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19
Q

School age oneumonia causes

A

S pneumoniae
S aureus
GAS
M pneumoniae

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20
Q

Intussusception definition

A

Invagination of proximal bowel into distal segment

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21
Q

S+S intussusception

A
  • Paroxysmal severe colicky pain with pallor
  • Pale around mouth, draw up legs
  • Recovery but increased lethargy between episodes
  • Vomit may be bile stained
  • Sausage shaped palpable mass
  • Redcurrant jelly = blood stained mucus
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22
Q

Mx intussucpetion

A

Fluid resus
Air insufflation
Surgery

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23
Q

meckels diverticulum

A
  • remnant of omphalomesenteric duct
  • 2 feet from IC valve, 2 inches long
  • rectal bleeding = painless
    99 t scan
    surgery
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24
Q

Pyloric stenosis S+S

A

Non bilious vomiting after feed, increasing in frequency and forcefullness until projectile

  • Keep feeding
  • Gastric peristalsis
  • Pyloric mass = palpable during feed in RUQ
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25
Pyloric stenosis Ix
- Test feed with NG tbe to aspirate stomach and exam | - USS
26
Mx pyloric stenosis
- Fix acid base electrolyte imbalances before surgery | - Pyloromyotomy
27
GORD definition
Involuntary passage of gastric contents into the oesophagus
28
Aetiology GORD
functional immaturity of lower oesophageal sphincter = inappropriately relaxed
29
RF for GORD
Prematurity CP Obesity HH
30
S+S GORD
- Faltering growth from severe vomiting - Oesophagitis - Aspiration, wheezing, hoarseness - Dystonic neck posturing - Apnoea
31
Ix GORD
- Ph monitoring - Endoscopy - Barium swallow
32
Mx GORD
- Feed thickening - PPI - Antacid, H2 blocker - Fundoplication
33
Kawasaki definition
Systemic vasculitis with a predisposition to involving coronary arteries
34
Diagnostic criteria Kawasaki
Fever >5 days and 4/5 of - Bilateral, bulbar, non purulent conjunctivitis - Changes in lips/oral mucosa = cracked, strawberry tongue, erythema - Changes in extremities = Oedema, erythema, desquamination - Polymorphous rash - Cervical lymphadenopathy
35
Ix Kawasaki
- CLinical findings - High inflam maekers - Platelet count rises in 2nd week
36
Mx kawasaki
- IVIG 2g/kg 12hr apart - IV methylprednisolone and infliximab - Aspirin - Antiplatelet agents of risk of thrombus - Cardiology follow up
37
Measles features
- Temperature - Rash = behind ears to body - Kolpik spots - Conjunctivitis and coryza - Cough and malaise
38
Measles treatment
- Supportive | - Immunocompromised = ribavirin
39
Nephrotic syndrome definition
- Proteinuria, hypoalbuminaemia, oedema
40
Nephrotic pathophysiology
- Podocytes flattened so allow leaking
41
S+S nephrotic syndrome
Oedema = initially on waking, periorbital - Ascites, labial and scrotal swelling - Pleural effusions and breathlessness
42
Nephrotic Ix
- Urinalysis - Microscopy - Serum albumin - U+E/creatinine
43
Nephrotic Mx
- Oral corticosteroids (60mg/m2 per day pred) --> 4 weeks change to 40 on alternate days - Resistant = diuretics, salt restriction, NSAIDs
44
Nephrotic indications for biopsy
- <12 months or >12 years - Increased BP - Macroscopic haematuria - Impaired renal function - Decreased C3/C4 - Failure to respond after 1 month daily steroid therapy
45
S+S VSD
- Small = asymptomatic - Pan systolic murmur lower left sternal edge - HF after 1 week if large (diuretics and captopril) - Tachycardia, pnoea and hepatomegaly
46
4 cardinal features of TOF
- Large VSD - Overriding aorta - Right ventricular hypertrophy - Sub pulmonary stenosis causing RV outflow tract obstruction
47
pulmonary stenosis finding
ESM upper left sternal border
48
S+S TOF
- Cyanosis - Paroxysmal hyper cyanotic spells - Ejection systolic murmur
49
Definition of rheumatic fever
Multisystem autoimmune response to a group A strep infection
50
Major criteria for RF
- Subcutaneous nodules - Erythema marginatum - Chorea (sydenham) - Migratory arthrotos = ankles, knees, wrists - Carditis
51
Minor criteria for RF
- Fever - Polyarthralgia - Raised CRP/ESR - Prolonged PR need 2 makor or 1 major and 2 minor
52
Rheumatic Fever Mx
- NSAIDs - Glucocorticoids if severe carditis - Penixillin - Assess for emergency valve replacement
53
Direction of shunt in PDA
- Left to right | - Flow of blood from aorta to PA following fall in PV resistance after birth
54
S+S PDA
- Continuous murmur below left clavicle = machinery - Collapsing/bounding pulse - If large = poor growth, feeding difficulty, resp difficulty, tachypnoea - Thrill/gallop
55
S+S IE
- Sustained fever, malaise, raised ESR, unexplained anaemia or haematuria = suspect - Murmur/changing cardiac signs - Anaemia and pallor - Nail signs - Necrotic skin lesions - Splenomegaly - Neuro signs from cerebral infarction
56
Major Dukes criteria
- +ve blood culture = 2 seperate | - Evidence of endocardial involvement = +ve echo fidnings
57
Minor dukes criteria
- Predisposition - FEver - Vascular phenomena - Immunologic phenomena - Microbiological evidence - ECHO
58
IE treatment
- High dose enicillin and aminoglycoside | - 6 weeks OV
59
HSP definition
Small vessel non-granulomatous IgA leukocytoclastic vasculitis
60
RF HSP
- URTI - Winter - 2-11
61
HSP patho
Circulating IgA levels increase and IgG synthesis disrupted. IgG and A produce complexes that activate complement and are deposited in affected organs
62
HSP S+S
- Maculopapular purpuric palpable rash = legs, buttock, arms- Trunk spared - Oedema - Abdo colic pain (haem and melaena) - Arthralgia - Macrscopic haematuria - Severe = intussuception
63
HSP Tx
- Steroids | - Benign and self limiting = most recover in 8 weeks
64
Glomerulonephritis definition
Acute renal failure due to damage/injury of glomeruli
65
Causes GN
- Postinfectious - Vasculitis - IgA nephropathy - Goodpastures
66
S+S GN
- HTN --> seizures - Oedema - Reduced UO and volume overload - Haematuria and proteinuria
67
Ix GN
- Urine dip - Microscopy = RBC and protein casts - USS
68
Mx Gn
- Treat hyperK, HTN, acidosis, an hypoca
69
Poststrep GN
- Throat or skin infection - Low complement C3 levels return normal after 4w - Culture to prove
70
IgA GN
- Macroscopic haematuria | - URTI
71
Alport syndrome
- Abnormalities of collagen in BM - X linked recessive - Nerve deafness and ocular defects
72
Triad for nephrotic
- Heavy proteinuria - Low plasma albumin - Oedema
73
Vesicoureteric reflux
Ureters displaced laterally and enter directly into bladder rather than at an angle
74
Long term impact vesicoureteric reflux
- Urine returning to bladder from ureters after voiding = incomplete empyting = infection - Pyelonephritis - Bladder voiding rpessure = transmitted to renal papillae = renal damage
75
Triad of HUS
- Microangiopathic haemolytic anaemia - Thrombocytopenia - AKI
76
HUS S+S
- fever - abdo pain - lethargy - oliguria - haematuria - bruising - jauncide - confusion
77
Mx HUS
- IV fluids - Transfusions - DIalysis self limiting = most fully recover | - Early supportive therapy and dialysis
78
Colic
- Inconsolable crying - Drawing up knees - Excessive wind - Evening - Benign - >2 weeks = cows milk allergy
79
Toddlers diarrhoea
- 'Peas and carrots' - Well and thriving children - Adequate fat and fibre
80
Whooping cough
- Bordetella pertussis - 1 week of coryza develop paroxysmal or sporadic cough - Inspiratory whoop - Night worse - Vomiting - Red or blue in face and mucus - 3-6 weeks - PCR = marked lymphocytosis on blood film - Immunisation
81
Slapped cheek
- Erythema infectiosum - viraemic phase of fever, malaise, headache and myalgia - rash on face turns to lace like maculopapular rash on trunk and limbs
82
Candida
- May cause and often complicates napkin rashes - Erythematous - Skin flexures and may be satellite lesions - Topical antifungal
83
Breath holding episodes
- Toddler - Precipitated by anger - Goes blue then limp - Rapid recovery
84
Reflex anoxic seizures
- Toddler - Precipitated by pain - Stops breathing - Goes pale - Brief seizure - Rapid recovery
85
Epilepsy
- Same as adults so look at treatment
86
Juvenile myoclonic epilepsy
- Myoclonic seizures shortly after waking | - Throwing drinks or cornflakes
87
West syndrome
- Infantile spasms - 6 months age - Clusters of full body spasms - Prednisolone and vigabatrin
88
undescended testes
- 4% at birth - Preterm more common - Retractacile = can be manpulated but then retracts - Palpable = can feel but not manipulate - Impalpable = no testis felt Ix = USS, measure testosterone after HCG injections, laparosciopy Mx = surgery
89
testicular torsion
- Atypical presentation if young with lower or inguinal abdo pain of sudden onset - Surgical exploration mandatory
90
Definition haemolytic disease of new-born
- Caused by incompatibility between rhesus antigens on surface of RBCs of mum and foetus - Mum Rh- and baby Rh+. 1st pregnancy mum will become sensitised to RhD antigens so subsequent pregnancies = anti D abs cross placenta = haemolysis = anaemia and high bilirubin levels - DCT
91
Microcytic anaemia causes
Thalassaemia Anaemia chronic disease Iron def Lead poisoning Sideroblastic TAILS ```
92
Thalassaemia definition
- A thal = defect in A globin chains B thal = defect in B globin chains - Autosomal recessive
93
S+S thalassaemia
- Microcytic anaemia - Fatigue and pallor - Jaundice - Gallstones - Splenomegaly - Pronounced forehead - Poor growth
94
Thalassaemia diagnosis
- FBC = microcytic anaemia - Hb electrophoresis - DNA test
95
G6PD deficiency
- X linked - Neonatal jaundice - Acute haemolysis = fever, malaise, dark urine
96
diagnosis and Mx G6PD
- Measuring G6PD activity in RBC | - Avoid precipitants
97
Sickle cell inheritance
- Autosomal recessive - 1 abnormal = trait - 2 abnormal = disease - Sickle cell anaemia = homozygous
98
manifestations of SCD
- Anaemia and jaundice - Increased susceptibility to infection - Vaso-occlusive crises - Acute anaemia - Splenomegaly
99
SCD Mx
- Antibiotic prophylaxis - Folic acid - Hydration - Crises = analgesia, hydration, abx, exchange of transfusion
100
Haemophilia definition
- X linked recessive - A = FVIII deficiency - B = FIX
101
S+S haemophilia
- Recurrent spontaneous bleeding into joints and muscles | - Neonates = IC haemorrhage, prolonged bleeding from heel stick
102
Haemophilia management
- Recombinant FVIII or FVIX concentrate IV infusion if bleeding
103
VWD
- deficiency of VW factor = defective platelet plug formation - Autosomal dominant
104
S+S VWD
- bruising - prolonged bleeding - Mucosal bleeding
105
VWD Mx
- Desmopressin - VWF - factor VIII
106
ITP
- Caused by type 2 sensitivity reaction | - Antibodies target and destroy platelets
107
ITP presentation
- Under 10 - Recent viral illness - Bleeding and bruising - purpuric non blanching rash - DO an urgent FBC for platelet count
108
ITP Mx
- Prednisolone - IVIG - Transfusions - Platelets
109
Avoid in ITP
- Contact sports - Avoid IM injections - Avoid NSAIDs, aspirin and blood thinners
110
What leukaemia is most common in children
ALL
111
Presentation ALL
- 2-5 years - Malaise - Anorexia - Anaemia - Neutropenia - Pallor - Bruising, nosebleeds - Headaches, vomting, palsies
112
Ix ALL
- FBC (low Hb, TP) - Blood film - BM biopsy - CXR
113
Mx ALL
- Chemotherapy
114
Neuroblastoma Ix
- Raised urinary catecholamine levels | - USS and MRI = abdo mass
115
Neuroblastoma Mx
- Surgery if localised | - Metastatic = chemo
116
Wilms tumour
- Neuroblastoma | - From embryonal renal tissue
117
S+S Wilms
- Large abdo mass | - Anaemia, haematuria, HTN
118
Wilms Ix and Mx
- USS or CT/MRI = intrinsic renal mass | - Initial chemo followed by delayed nephrectomy
119
Bone tumour
- Osteogenic sarcoma more common than Ewing - Ewing in younger children - Male predominance - Bone pain - XR - Chemo and surgery
120
Retinoblastoma
- Malignant tumour retinal cells | - Csome 13 = susceptibility gene = dominant
121
S+S retinoblastoma
- white pupillary reflex to replace red reflex | - Squint
122
Ix and Mx retinoblastoma
- Enucleation if advanced - Chemo t shrink - Local laser treatment
123
Downs syndrome features
trisomy 21 - Hypotonia - Brachycephaly - Short neck - Short stature - Flattened face and nose - Prominent epicanthic folds - Upward sloping palpebral fissures - Single palmar crease
124
Downs syndrome complications
- Learning disability - Recurrent otitis media - Deafness - Visual problems - Hypothyroid - Cardiac defects - Leukaemia
125
Klinefelter syndrome
- Male has additional X csome = 47XXY
126
Mx klinefelter
- Testosterone injecitions | - MDT input
127
Turner syndrome
- Female has single X csome = 45X0
128
Turner features
- short stature - Webbed neck - High arching palate - Ptosis - Broad chest - Cubitus valgus - Underdeveloped ovaries - Late or incomplete puberty
129
Turners Mx
- GH therapy | - Oestrogen and progesterone
130
Features fragile X
- Intellectual disability - Long narrow face - Large ears - Large testiles - Hypermobile joints - ADHD - Autism - Seizures - Mitral valve prolapse
131
Prader Willi features
- Csome 15 = deletion - Insatiable hunger - Hypotonia - Hypogonadism - Soft fair skin - MH problems - Narrow forehead - Strabismus - Thin upper lip
132
Patau syndrome
- Trisomy 13 - Rocker bottom feet - polydactyly - cleft lip and palate - microcephaly
133
RDS definition
- Surfactant deficiency = lowers surface tension = alveolar collapse and inadequate GE
134
How to reduce risk of RDS
- Glucocorticoids gien antenatally to mother to stimulate foetal surfactant production
135
S+S RDS
- Within 4hrs of birth will get - Tachypnoea >60 - Laboured breathing with chest wall recession and flaring - Expiratory grunting - Cyanosis
136
RDS CXR
- Ground glass appearance and air bronchogram
137
RDS Mx
- Raised ambient oxygen | - May need continuous positive airway pressure or artificial ventilation
138
Necrotising enterocolitis
- Bacterial invasion of ischaemic bowel wall
139
S+S NE
- Stop tolerating feeds - Milk aspirated from stomach - Vomiting (bile) - Abdo distension - Blood in stool - Shock
140
NE XR
- distended bowel loops and thickening of bowel wall with intramural gas
141
Mx NE
- Artificial ventilation - Stop oral feeding - Broad spectrum abx - Parenteral nutrition - Surgery if perforation
142
Kernicterus
- Encephalopathy resulting from deposition of unconjucated bilirubin in basal ganglia and brainstem nuclei
143
What level to babies become clinically jaundiced
- 80 umol/l
144
Jaundice <24 hrs age
- Usually results from haemolysis | - Bili = unconjugated and can rise rapidly
145
Haemolytic disorders
- Rhesus haemolytic disease - ABO incompatibility - G6PD - Spherocytosis
146
Causes of jaundice 2 days - 2 weeks
- Physiological - Breast milk jaundice - Dehydration - Infection
147
Management of jaundice
- Phototherapy | - Exchange transfusion
148
Jaundice >2 weeks old
- Persistent neoneatal jaundice | - May be caused by biliary atresia
149
Meconium aspiration
- Asphyxiated infants may start gasping and aspirate meconium before delivery - Meconium = lung irritant = mechanical obstruction and chemical pneumonitis - Overinflated lungs with patches of collapse and consolidation - Pneumothorax - May develop persistant pulmonary HTN
150
S+S neonatal sepsis
- Fever - Poor feeding - Vomiting - APnoea and bradycardia - RDS - Abdo distension - Jaundice - Neutropenia - Glycaemia - Shock - Seizures
151
Group B strep infection RF
- PPRM - Maternal fever in labour - Maternal chorioamnionitis - Previous infected infant - Prophylactic abx given
152
Hypoglycaemia symptoms
- Jitteriness - Irritability - Apnoea - Lethargy - Drowsiness - Seizures
153
Ix neonatal seizre
- Cerebral USS | - Hypoglycamia and meningitis need to be ruled out
154
Gastroschisis
- Bowel protrudes through defect in anterior abdominal wall adjacent to umbilicus - Risk of dehydration and protein loss - Clingfilm to minimise fluid and heat loss - NG tube - IV dextrose - Colloid support - Surgerhy
155
Foetal problems associated with maternal diabetes
- Congenital malformations - IUGR - Macrosomia
156
Pathophysiology T1DM
- Destruction of pancreatic B cells by autoimmune process
157
S+S DM1
- Polydipsia - Polyura - Weight loss - Candida - Sking infection - DKA - 2 peak ages = age 5-7 and before puberty
158
Ix DM1
- Random glucose >11.1 - U+E, ketones, urine, blood pH, autoantibodies - Fasting blood glucose >7
159
Mx DM1 insulin changes on age
Requirement for insulin changes on age - Childhood = 0.5-1 u/kg/day - Puberty = 1.2 - 2 kg/day - post puberty = 0.7-1.2 u/kg/day Target = 4-10mmol/l
160
S+S DKA
- Acetone breath - Vomiting - Dehydration - Abdo pain - Kussmaul - Hypo shock - Drowsy
161
Definition DKA
- Hyperglycaemia >11mmol/l - pH <7.3 - Bicarb <15 - Urinary ketones
162
DKA Ix
- Glucose - Ketones >3 - ABG - U+E, creatinine - Cardiac monitor - Weight - Lactate
163
ABG results for mild, moderate and sever DKA
- Mild = pH >7.2 or HCO3 10-15mmol/l - Moderate DKA = pH 7.1-7.2 or HCO3 5-10 - Severe DKA = pH<7.1 or HCO3 <5
164
Mx DKA
- Emergency measures = if shocked give bolus 10ml/kg 0.9% NaCl and cosider abx - Fluids = no shock give initial 10ml/kg bolus +rehydration fluids + maintenance fluids - Insulin 0.05 units/kg/hr
165
Complications DKA
- Cerebral oedema | - Rapid correction can cause rapid water shift from extra to intra = oedematous brain
166
Mx hypoglycaemia
- IV glucose 2ml/kg of 10% dextrose | - Sweating, pallor, CNS
167
Congenital hypothyroidism S+S
- Guthrie positive - Prolonged neonatal jaundice - Poor feeding - Constiptation - Increased sleeping - Reduced activity - Slow growth and development
168
Acquired hypothyroidism S+S
- Fatigue and low energy - Poor growth - Weight gain - Poor school performance - Constipation - Dry skin and hair loss
169
CAH definition
- Underproduction of cortisol and aldosterone and overproduction of androgens from birth - AR = congenital deficiency of 21 hydroxylase
170
CAH pathophysiology
- 21H converts progesterone to aldosterone and cortisol. lack therefore means extra progesterone converted to testosterone = low ald and high test
171
S+S CAH
- Ambiguous genitalia, enlarged clitoris - Severe = after birth hypona, hyperka, hypogly - Poor feeding - Vomiting and dehydration - Arrhythmia older - Tall - Facial hair - Absent periods - Small testicles, large penis - Hyperpigmentation
172
Adrenal crisis S+S
- 1-3 weeks old - Vomiting - WL - Floppy - Circulatory collpase Mx - Hydrocortisone - Saline - Glucose
173
CAH Ix
- Low plasma Na - High plasma K - Met acodosis - hypo
174
Mx adrenal
- Hydrocortisone to replace cortisol - Fludrocortisone to replace aldosterone - Surgery
175
RF for OM
- 6-16 months - Male - Passive smoking - Bottle feed - Craniofacial abnormalities suppurative = mucopurulent discharge Effusion = grey TM, loss of light and fluid behind TM
176
S+S OM
- Severe pain - Systemic features - Coryzal - Bright red and bulging tympanic membrane, loss of reflection - Test facial nerve function
177
OM causative
- S pneumonia | - RSV, rhinovirus
178
Mx OM
- Usually spontaneous recovery - ABX shorten pain but dont reduce hearing loss - <2yo = antibiotics (amox, erythro)
179
OM complication
Mastoiditis - Necrosis, subperiosteal abscess - Boggy, erythematous swelling behind ear - IV Abx
180
OM with effusion
- Glue ear = result of recurrent ear infection - Viscous inflammatory fluid build up - Eardrum dull, retraces, visible fluid level - Asymtpomatic apart from hearing loss - Spontaneous recovery - Grommets if not recovering
181
S+S asthma
- Suspect in any child with wheeze - Wheeze, cough and breathlessness worse at night and morning - Interval symptoms
182
Mx asthma
1. SABA for all >5 2. ICS 3. LRTA 4. LABA 5. MART <5 2. ICS 8 week trial 3. LRTA
183
Moderate asthma attack
- Talk - Sats >92 - PF >50% • RR - <40 for 1-5 - <30 for 5-12 - <25 for 12-18 • HR - <140 for 1-5 - <125 for 5-12 - <110 for 12-18 • SABA, prednisolone, monitor
184
Severe asthma attack
Too breathless to talk •O2 sats <92 for <12 •Peak flow 33 – 50% •RR - >40 1-5 - >30 5-12 - >25 12-18 • HR - >140 1-5 - >125 5-12 - >110 12-18 • Oxygen, SABA, prednisolone • Consider ipratropium
185
Life threatening asthma attack
- Silent chest, cyanosis - Poor resp effort - Exhaustion - Arrhhythmia, hypotension - PF <33 - )2 <92 - Oxygen, SABA, Pred, neb ipratropium
186
Epiglottitis
- Similar clinical to croup - H influenza - Ill child, high fever - Drooling unable to swallow - Soft stridor EMERGENCY - O2 and neb adrenaline - Anaesthetist, paeds and ENT - Keep calm as crying bad
187
CF definition
- AR = mutation in CF transmembrane condictance regulator fene
188
CF Pathophysiology
- Multisystem disorder = results from abnormal ion transport across epithelial cells - CFTR gene encoded CFTR protein = Cl channel. Cl is driven against its concentration gradient using ATP - Airways = reduction in airway surface liquid layer  impaired ciliary function and retention of mucopurulent secretions which are prone to infection - Defective CFTR = dysregulation of inflammation an defence against infection = Intestine = thick viscid meconium  meconium ileus in 10-20% - Pancreatic ducts = blocked with thick secretions  pancreatic enzyme deficiency and malabsorption - Sweat gland abnormal function  excessive concentration of Na and Cl in sweat
189
CF S+S
- Meconium ileus - Prolonged neonatal jaundice - Recurrent infections - Malabsorption, steatorrhea - Bronchiectasis - Persistent cough - DM - Cirrhosis and portal HTN - hyperinflation, coarse creps inspiration and expiratory wheeze , clubbing
190
CF Ix
- Sweat test - Genetic testing - CXR = hyperinflation, bronchial dilation, cysts, linear shadows
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CF Mx
- Nebulized mucolytics = DNase and hypertonic saline - ABx - Lactulose - Pancreatic enzyme replacement - CFTR modulators - Physio - high calorie high fat diet
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JIA definition
- Persistent joint swelling of >6 weeks presenting before 16 YO in absence of infection or other cause - Oligo = up to and including 4 - Poly = more than 4
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S+S JIA
- Morning joint stiff - Pain - Young = intermittent limp or mood deterioration - Swelling - Gradual onset - Non use - Rash, fever, WL
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Ix JIA
- Exclusion in children <16 - FBC, CRP/ESR, infection screen, Rh, ANA - Imaging - Early XR = soft tissue swelling, juxta-articular osteopenia - Late XR = joint space narrowing and erosions - Gadolinium enhanced MRO GS for synovitis
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Mx JIA
- NSAIDs - Joint injections = 1st line in oligo - Methotrexate - Corticosteroids if severe - Biologics = cytokine modulators
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JIA complications
- Chronic anterior uveitis = refer to opthalmology - Joint contractures and erosions
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DDH RF
- FHx - Female - Breech
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DDH S+S
Infant - Asymmetrical gluteal folds - Limited abduction - LL discrepancy - Galeazzi sign Older - Limp - +ve trendelenburg - Bilateral dislocations = exaggerated lumbar lordosis and limited hip abduction
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Ix DDH
- Ortolani and Barlow = NIPE - <6 months USS >6 months AP pelvis radiograph = shallow acetabulum, increased ondex, hypoplastic femoral head
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DDH Mx
<6 months - Pavlik harness 6-18 months - Manipulation and closed reduction and plaster cast 18-24 months - Trial of closed reduction +/- pelvic osteotomy and cast 2-6 years - Open reduction +/- femoral shortening +/- pelvic osteotomy and cast
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Perthes definition
- Idiopathic avascular necrosis of capital femoral head due to interruption of blood supply
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RF Perthes
- Boys - 5-10 - Obesity - Trauma - Endocrine/metabolic
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S+S Perthes
- Insidious presentation - Limp, hip or knee pain - Mild intermittent anterior thigh/groin/knee pain - Bilateral - Painless limp
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Ix perthes
- Early XR = normal - Later XR = increased density in femoral head = fragmented and residual deformity A = no loss of height of lateral 1/3 B = up to 50% loss of height C = >50% loss of height - Technetium 99 scan
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Perthes on examination
Look - Proximal thigh atrophy - Mild short stature - Limp/Trendelenberg/antalgic gait Feel - Effusion - Groin/thigh tenderness Move - Decreased hip ROM with spasm
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Perhtes Mx
- Physio - Rest and walking aids - NSAIDs - Surgery - Local self healing disorder
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Osteomyelitis definition
- Infection of metaphysis of long bone
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Causative organisms Osteomyelitis
- Staph aureus - Neonates = GBS - <2 HI - >2 gram +ve
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S+S osteomyelitis
- Low grade pyrexia and malaise - Markedly painful immobile limb - Tender, warm swelling - POM - Infants = swollen and ROM
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Ix osteomyelitis
- Cultures - WCC, CRP and ESR raised _ XR = early normal, late = metaphyseal rarefaction, destructive changes after 10 days
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Mx osteomyelitis
- IV Abx 2 weeks then oral for 4 | - Drainage and debridement
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Septic arthritis S+S
Knee>hip>ankle - Erythematous warn tender joint - Reduced ROM - Hold limb still - Joint effusion in peripheral joints - 50% dont have fever
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SA Ix
- Bloods = increased WCC - Cultures - XR = joint space narrowing and erosive changes - Aspiration - USS - MRI
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SA Mx
- IV Abx after aspirate taken for up to 3 weeks then oral 4-6 weeks - Surgery = irrigation and debridement - Splnitage - Physio
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Type 1 osteogensis imperfecta
- AD - Fractures in childhood - Blue sclera
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Type 2 OI
- Lethal | - Multiple fractures before birth
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S+S rickets
- Rachitis rosary - Horizontal depression lower chest - Bowed legs - Softening of skull vault - Delayed closure fontanelle - Hypotonia
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XR rickets
- Cupping and fraying of metaphyses - Widened growth plate - Joint widening - Bowing of diaphysis - Thickening and widening of epiphysis
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Transient synovitis
- Follows or accompanied by viral infection - Sudden onset in pain or a limp - No pain at rest - Decreased ROM - May refer to knee - Afebrile
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Slipped upper femoral epiphysis
- RF = AA, endo, obese - Groin thigh or knee pain - Antalgic gait - Limited hip flexion and abduction - Thigh atrophy
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Malrotation
- 1-3 days of life present with obstruction from Ladd bands obstructing duodenum or with volvulus - Bilious vomiting, abdo pain, tender from peritonitis - GI contrast study needed if bilious vomiting - Surgical correction
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Biliary atresia
- Section of bile duct narrowed or absent - Cholestasis and prevented excretion conjugated bili - Present after birth = jaundice (persistent) and high conj bili - Surgery = kasai portoenterostomy - Transplant liver
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Pathophysiology cows milk allergy
IgE mediated - Type 1 hypersensitivity - CD4 + TH2 cells stimulate B to produce IgE abs = histomine and cytokines Non IgE - T cell activation againt CMP
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IgE CMP S+S
- Acute onset - Pruritus, erythema, urticaria, angio-oedema - N+V - Colick, diarrhoea
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Non IgE CMP S+S
- Delayed - Pruritus, erythema, atopic eczema - GORD - Loose stools - Blood/mucus - Abdo pain, food refusal, pallor, tiredness
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Mx CMP allergy
- Avoidance - MAP guideline - Hydrolysed formula or AA formula
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UC histology and radiology
- Crypt abscesses, mucosal inflammation, architectural distortion, crypt loss - Mucosal ulceration, haustration loss, colonic narrowing
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S+S UC
- Rectal bleed - Diarrhoea - Colicky - Weight loss and grwoth failure
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MX UC
- Amino salicylates for induction and maintenance - Aggressive = steroids - Surgery
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Crohns patho
- Mouth to anus - Skip lesions - Transmural infalmmation = deep ulcers and fissures - Non caseating granulomatous inflamation
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Crohns S+S
- Growth failure, puberty delayed - General ill health - Abdo pain, diarrhoea, blood, weight loss - Oral lesions - Uveitis
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Crohn's Ix
- Bloods PANCA - MC+S - Calcoprotein - Endoscopy and radiology (crypt abscesses, transmural inflammation, cobblestones)
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Crohns Mx
- Mild = oral 5 ASA dimers - Moderate = pred to induce remission - Maintenance = Immunomodifiers
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Crohn's vs UC
Crohn's - Mouth to anus - Transmural - Discontinuous - Granuloma and recr=tal sparing - Fissures, fistula, strictures - perianal disease UC - Colon only - Mucosal - Continuous - no granuloma and no rectal sparing - Abscesses and strictures rare - primary sclerosing cholangitis
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Gastroenteritis - general Ix
- Stool sample if blood, septicaemia, immunocompromised | U+E = IVF if hyperna
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When to consider DD for gastroenterieis
- Temp >38 if <3m - Tachy - GCS altered - Meningism - Blood/mucus - Green vomit - Distension or guarding
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Mx gastroenteritis general
- no dehydrated = feeds, fluids dehydrated - fluid deficit replacement and maintenance - IV if shock - ORS 50ml/kg over 4 hrs
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Classical form coeliac
- 9-24 months - Malabsorption - FTT and weight loss - Loose stools and steat - Anorexia - Abdo pain and distension - Behaviour change - Crypt hyperplasia and villous atrophy
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Atypical form coeliac
- No intestinal sx - Extra sx = OP, neuropathy, anaemia, infertility - +ve serology - limited abnormalities
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Hirschprung's disease
- Absence ganglion cells in hindgut = ansence of coordinated peristalsis - Neonates, fail mec in 24hrs, abdo distension and bile vomit - older = chronic constipation, abdo distension, growth faltering
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Hirschprung Ix and Mx
- DRE = gush stoll and flatus - AXR = distal intestinal obstruction - Biopsy - Colostomy and anastomosis = Swenson procedure
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When to biopsy for hirschprung
- delayed mecomium 48hrs - constipation since first few weeks of life - chronic distension and vomiting - FHx - Faltering growth
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Diagnostic criteria for constipation
- <3 complete stolls a week - hard large stool - rabbit droppings - overflow soiling
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Constipation management
1. laxative (macrogol) and diet 2. stimulant laxative (senna) 3. lactulose/softener
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Measles
- Temperature - rash = behind ears, whole body - Koplik spots = white on inside cheeks - Conjunctivitis, cough, malaise - Supportive treatment - immunocompromised = ribavarin
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Mumps
- Fever, malaise,parotid gland swelling - 1 side face swollen - earache - plasma amylase elevated
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Rubella
- Maculopapular rash, lymphadenopathy Complications - arthritis - encephalitis - thrombocytopenia - myocarditis
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Chicken pox
- VZV - vessicles on trunk/face and spread outwards - lesions scab over = no longer infectious - fever, itch, malaise - 5 day exclusion - aziclovir if serious - VZIG prophylaxis
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Impetigo
- staph or strep skin infection - lesions on facem neck and hands begin as erythematous papules - rupture of vesicles = honey coloured crusted lesions - topical hydroperoxidde abx - fluclox if severe
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toxic shock
- s aureus or group a strep - fever over 39 - hypotension - diffuse erythematou macular rash - organ dysfunction - thrombocytopenia, coagulopathy, abnomral LFTs - manage shock, debridement - cef with clindamycin - IVIG to neutralise circulating toxin
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Scalded skin syndrome
- Exfolaitive staph toxin - fever, malaise - Purulent crusting around eyes,nose, mouth - widesread erythema and tenderness - areas seperate ong ente oressure - Fluclox, analgesia, hydration
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HIV S+S
- lymphadenopathy - parotid enlargement - recurrent bacterial - candidiasis - chronic diarrhoea - pneumonitis
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HIV Ix
- >18m = detect antibodies | - <18m = transplacental maternal IgG HIV antibodies so positive test shows exposure not infection
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HIV Mx
- prophylactic co-trimoxazole if low CD4 | - Immunisations
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TB S+S
Asymptomatic - Minimal or no S+S - Positive Mantoux or interferongamma release assay = give chemoprophylaxis Symptomatic - Lung lesion plus LN = Ghon/primary complex - Fever - Weight loss - Cough - CXR changes 4-8 weeks - Febrile illness - Erythema nodosum - Phlyctenular conjunctivitis 6-9 months - Progressive healing of primary complex - Effusion - Cavitation - Coin lesion on CXR - Regional LN may obstruct bronchi - Regional LN may erode into bronchus or pericardial sac - Miliary spread REACTIVATION = tuberculous meningitis
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TB Ix
- Sputum on 3 consecutive = ZN stain for AFB - Urine, LN, CSF - CXR - Mantoux skin test
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Mx TB
pulmonary - 2 months RIPE - 4 months RI miliary - 3 months RIPE - 12-18 months RI
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EBV
- Causes mono - fever, malaise, tonsilitis, petechia palate, maculopapuar rash - +ve monospot test - Symptomatic treatment
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Febrile seizures
- 2-5minutes, high fever, underlying illness | - manage underlying source of infection
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Hypospadias
- Urethral opening proximal to normal meatus - Hooded dorsal foreskin - Chordee - No circumsision - surgery before 2yo
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HSP
- Non granulomatous IgA vasculitis - URTI - Purpura legs, bum, arms, symmetrical and trunk spared - macroscopic haem or mild protien - angioeoedema - Mx = rest, analgesia, hydrate, steroids
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Spina bifida occulta
- No herniation neural tissue | - dermal sinus, dimple, lipoma, hairy naevus
263
Spina bifida meningocele
- Herniation fluid and meninges only skin covering - surgical closure
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Spina bifida myelomeningocele
- Herniation spinal neural tissue - adjacent spinal cord abnormal - flaccid paralysis below lesion, incontinence, urinary tract dilatation - surgical closure and hydrocephalus drainage
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Mx eneurisis
- fluid restriction before bed and empty bladder -star chart - eneurisis alarm desmopressin
266
Mx eczema
- Mild = 1% hydrocortisone - Mild - moderate = clobetasome 0.05% - moderate to severe = monentason furouate
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S+S dka
Dehydration Kussmaoul Smell ketones lethargy, drowsiness
268
DKA biochemistry
``` - Glucose >11 Acidameia = <7.3 - Ketones >3 Mild = pH <7.3, 5% dehydration Mod = <7.2 and 7% dehydration Sev = <7.1 10% dehydration ```
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IV Mx DKA
- 10ml/kg fluid bolus over 60 minutes - Correct fluid deficit over 48hrs - 0.9% NaCl with 20mmol KCL in every 500ml - Insulin 0.05 or 0.1 units/kg/hr 1-2hrs after fluids
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Rickets biochemical picture
- Reduced serum Ca and Phosphate | - Raised serum ALP
271
Esotropia
Inward positioned squint (affected eye towards nose)
272
Exotropia
- Outward positioned squint (affected eye towards ear)
273
Hypertropia
Upward moving affected eye
274
Hypotropia
Downward moving affected eye
275
causes of squint
- isiopathic - hydrocephalus - cerebral palsy = space occupying lesions - trauma
276
Hirschberg's test
- pen torch from 1m - observe reflection of light source on cornea - Deviation from centre = squint
277
Cover test
- Cover 1 eye and ask patient to focus on an object in front - Move cover to opposite eye and watch movement of other eye - If move inwards it has drifted outwards when covered = exotropia - If moves outwards it has drifted inwards when covered = eso
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Audiometry
- <3y tested for basic response to sound - Older = specific tones and volumes - Audiometry recorded on audiogram = identify conductive and sensorineural hearing loss
279
Audiogram
- Establish the minimum volume required for the patient to hear each frequency - Air and bone conduction tested seperately - Normal hearing = between 0 and 20 DB
280
Sensorineural hearing loss
- Air and bone more than 20 DB
281
Conductive hearing loss
- Bone - normal | - Air = greater than 20db
282
what hearing test for 6-18m
distraction
283
what hearing test for 3y+
pure tone audiomentry
284
what hearing test for 24+m
performance test
285
what hearing test for 6-30m
visual reinforcement
286
Mixed
- Air abd bone more than 20 | - Difference of 15db between the 2
287
Mx ADHD
- Methylphenidate (ritalin) - Dexamfetamine - Atomoxetine
288
IM adrenaline doses >12y
- 500mcg IM (0.5ml)
289
Adrenaline age 6-12y
300mcg IM
290
adrenaline 6m-6y
- 150mcg
291
adrenaline <6m
100-150mcg
292
roseola infantum
- Herpes simplex 6 - after fever subsides - SE = febrile seizures
293
Nephrotic complications
- Hypercholesterolaemia = cholesterol correlates inversely with serum albumin - Thrombosis - Infection - Hypovolaemia
294
amblyopia
- Defective visual acuity | - Persists after correction of refractive error and removal of any pathology
295
<3m S+S UTI
- FTT - Irritable, lethargy - Fever, vomiting - Poor feed - <3m suspect UTI = refer = abx, sepsis screen, mc+s
296
>3m S+S UTI
- Continence change - Vomiting - Frequency,dysuria - Pain and loin tenderness - Poor feed
297
when do you need a urine sample
- Fever >38 - S+S - <3m - Alternative site infection but stays unwell
298
Mx UTI
- >3m pyelo = cef or coamox - >3m cystisis = triemth or nitro - <3m = IV cef
299
Atypical UTI
- Non e coli - increased creatinine - Fail respond 48hrs - mass felt ix - MCUG - DMSA scan - USS
300
Impetigo
- Staph aureus - Off school until dry and scabbed or 48hrs abx - Topical 1% hydrogen peroxide 5 days if non-bullous or uncomplicated - 2% fusidic acid or mupirocin for 5 days if hydro not tolerated or around eyes or ineffective
301
Turners heart issues is...
bicuspid aortic valve or coarctation aorta
302
coarctation of aorta findings
- crescendo decrescendo murmur in upper left sternal border
303
CF inheritance
Autosomal recessive
304
scarlet fever caused by
- strep pyogenes = GAS - sanpapaer rash, strawberry tongue, circumoral pallor - mx = phenoxymethylpenicillin
305
what are the live vaccines
- MMR - BCG - Chickenpox - nasal flu - rotavirus
306
when do children get MMR
- 1 year old - 3yr and 4m
307
what is in the 6 in 1
- diptheria - tetanus - pertussis - polio - Hib - hep b
308
when do children get Men B
- 16 weeks - 1 year
309
Formula for weight if not given
(Age + 4) X 2
310
Maintenance fluids
- 1st 10kg = 100 - next 10kg = 50 - every other kg = 20 0.9% nacl + 5% glucose
311
deficit fluids
% X 10 X Kg
312
Bolus
- 10 mls/kg 0.9% NaCl
313
DKA shocked fluids
10ml/kg bolus saline over 15 minutes and dont subtract later
314
DKA not shocked
10ml/kg bolus saline over 30 minutes and do subtract later
315
fluid therapy DKA
{(deficit - unshocked bolus)/48} + maintenance hrly - 0.9% saline and 20mmol K in every 500ml bag
316
NICE guidelines for suspect cerbral palsy
- not sitting by 8 months - not walking by 18m - early hand prefference before 1y - toe walking
317
fraser guidelines
- cant be persuaded to inform parents - understands advice or tx - suffer if do not receive - best interests - continue sex without treatment