Paediatric FC from ppt Flashcards

Question

what are the refractive errors seen in childhood

Answer 1

1. hypermetropia 2. myopia 3. amblyopia

Answer 2

hypermetropia - long sightedness (MC) myopia - short sightedness (uncommon and less likely to elad to permenant visual changes) correct early to avoid amblyopia - irreversible damage to vision

Answer 3

also known as lazy eye potentially permanent loss of visual acuity in an eye that has not received a clear image usually affects one eye

Answer 4

any interferemce with visual development: squint refractive erros ptosis cataract

Answer 5

glasses to correct visual impairment patching of good eye to force lazy eye to work early treatment essential - after 7 years unlikely to improve considerable support to children and parent - children do not like having eye patched

Answer 6

loss of red reflex (from cataract) white reflex in the pupil (retinoblastoma, cataract, ROP) not smiling responsivley by 6 weeks lack of eye contact with parents random eye movements failure to fix and follow nystagmus squint photophobia

Answer 7

conservative - glasses, orthoptic exercises to improve eye muscle control surgery: strengthen procedure - resection weakening procedure - recession of muscle on side the eye goes towards esotropia (eye pointing inwards) - strengthen lateral rectus by resection, recession of the medial rectus exotropia - opposite botox injections - paralyse muscle that is pulling the eye in a certain direction

Answer 8

appears to daydream sits near the TV and turns the volume really loud watches the speaker's face closely for clues misunderstands/slow in responding answers questions incorrectly soft/fuzzy speech doesn't turn immediately when name called aggressive alowly schoolwork/grades start to get worse

Answer 9

conductive hearing loss - an obstruction in the ear canal preventing sound from getting through (often reversible) sensorineural hearing loss - nerve damage (progressive, never reversible)

Answer 10

most common - congestion behind the eardrums (with a cold) glue ear ear wax middle ear infection peforated ear drum structural abnormality of the outer ear - certain syndromes

Answer 11

Down's syndrome craniofacial syndromes cleft palate

Answer 12

better hearing through bone conduction

Answer 13

most are self-limiting (inner ear infection/cold) ENT referral - insertion of grommets to help drain excess fluid out of the middle ear hearing aids if they have a permanent cause for the hearing loss

Answer 14

unknown in many cases genetic / syndromal perinatal cause - trauma, infection, hypoxia at birth congenital infections - rubella, CMV meningitis - pneumococcus can cause ossification of the cochlear premature babies - increased risk

Answer 15

hearing is not better through bone on audiology - hearing loss worse in higher frequencies

Answer 16

hearing aids cochlear implants aim = raise the level of hearing so that as much speech is audible as possible

Answer 17

profound hearing loss (>90 decibles) high frequency bilateral hearing loss meningitis hearing loss

Answer 18

developmental delay - particularly in speech and language behavioural problems - too loud or too quiet impact on education impact on friendship and social life impact on emotions/psychosocial impact

Answer 19

paediatrician PT OT SALT dietician nurses health visitor psychologist family therapist social worker key worker teachers and school nurses may also be involved

Answer 20

help liaise between home and school and the childs care needs assess the child's functional ability and need to provide therapy where needed to provide psychosocial support to the family to ensure health needs of the child are met

Answer 21

trisomy 21 chromosomal abnormality in which there are three copies of T21, rather than 2 copies leads to specific learning difficulties and dysmorphic features

Answer 22

increasing maternal age

Answer 23

1. non-dysjunction - 94% 2. unbalanced Robertsonian translocation - 4% 3. gonadal mosaicism - 1% (milder phenotype)

Answer 24

an error in meiosis 1 pair of chromosomes 21s fail to seperate - one gamete has 2 21s and other has none fertilisation of gamete w/ 2 = zygote with trisomy 21 parents chromosome do not need to be examined related to maternal age 47 chromosomes in karyotype

Answer 25

extra copy of chromosome 21 joined onto another chromosome - usually C.14 these children will have 46 chromosomes on a karyotype - but 3 copies of 21 material (one portion tacked onto 14) parental chromosomes analysis is needed - one parent is a carrier in 25% of cases translocation carriers have 45 chromosomes on karyotype (one of the 21s is in wrong place)

Answer 26

flat bridge of nose flat occiput wide space between the eyes small mouth and hence apparently large tongue (pseudo macroglossia) single palmar crease eyes slanting down and inwards

Answer 27

complete atrioventricular septal defect - ECHO all T21 babies hypothyroidism duodenal atresia hypotonia later in life - Alzheimer's

Answer 28

risk score - serum markers and nuchal translucency diagnostic testing if high risk score - amniocentesis or chorionic villus sampling

Answer 29

MDT team involvement cardiology endocrinology childhood disability services SALT physio special education needs school liasion between school, healthcare and family imperative

Answer 30

low birthweight prominent occiput small mouth and chin short sternum flexed, overlapping fingers rocker-bottom feet cardiac and renal malformation microcephaly 90% die in first year

Answer 31

structural defect of brain (single lobed) scalp defects small eyes (microopthalmia) cleft lip and palate polydactyly cardiac and renal malformations not compatible with life

Answer 32

often antenatally - abnormalities detected on USS, risk score comes back high for trisomy prenatal diagnosis - amnio or CVS karyotype genetic analysis confirms diagnosis at birth

Answer 33

a chromosomal condition in which females only inherit one copy of the X chromosomes, rather than 2 copies should be 46, XX Turner's = 45, X or 45 XO

Answer 34

female short stature webbed neck infertility/primary amenorrhoea delayed puberty wide spaced nipples

Answer 35

coarctation of the aorta

Answer 36

clinical suspicion diagnosis confirmed on karyotypin (45, XO)

Answer 37

growth hormone therapy oestrogen replacement - develop secondary sexual characteristics at time of puberty infertility - IVF

Answer 38

a genetic condition in which males inherit an extra copy of the X chromosome - karyotype = 47, XXY (should be 46, XY)

Answer 39

male Tall stature infertility - adult life delayed puberty - lack of pubic hair gynaecomastia in adolescence

Answer 40

testosterone replacement - 1 injection/month for life promotes development of sexual characteristics

Answer 41

an inherited form of learning disability due to a trinucleotide repeat expansion on the X chromosome affects males more thna females as they have protection from their other X chromosome females have a milder phenotype

Answer 42

'cocktail personality' - happy, bouncy children macrocephaly large ears learning difficulties/autism joint laxity large testes hypotonia mitral valve prolapse

Answer 43

FISH testing (fluorescence in situ hybridisation) - to look at the content of the cells

Answer 44

abnormal branchial arch development leading to problems with the heart, thymus and palate

Answer 45

CATCH 22: cardiac abnormalities thymus hypoplasia (T-cell dysfunction => primary immune def.) cleft palate hypocalcaemia 22 - defect - chromosome 22 material deletion

Answer 46

truncus arteriosus tatralogy of fallot interrupted aortic arch coarctation vascular ring around trachea => stridor

Answer 47

T-cell count CXR - any pt with recurrent infections to look for thymus anyone with congenital cardiac disease - test for diGeorge

Answer 48

look like the boy version of Turner's short stature webbed neck ptosis hypothyroidism pulmonary stenosis hypogonadism

Answer 49

a connective tissue disorder caused by fibrillin deficiency

Answer 50

arachnodactyly aortic dilatation => aneurysm => dissection lens dislocation long arms and legs high arched palate lax/hypermobile joints

Answer 51

ECG - check for aortic dilatation - fix to prevent dissection

Answer 52

Elastin defect so affects collagen - skin etc

Answer 53

stretchy skin able to touch thumb to radius side of their arm excessive bruising (weak collagen in blood vessels) tissue fragility brain aneurysms 'cigarette paper scars' - shiny thin scars on the skin poor healing

Answer 54

auto dom auto rec X-linked Y-linked

Answer 55

adult polycystic kidney disease familial hypercholesterolaemia Marfan's syndrome Huntington's disease some cancers - BRCA

Answer 56

MC = non-paternity new mutation gonadal mosaicism

Answer 57

1 in 4 (25%)

Answer 58

2 in 3 carrier risk for unaffected siblings (take away possibility of them being affected)

Answer 59

consanguinous parents especially in families with many generation of consanguinity - can look like an auto dom pedigree for a rec condition

Answer 60

dom = structural protein defects rec - affects metabolic pathway

Answer 61

NO male to male transmission (2 consecutive generational males squares shaded on a pedigree, it is not X linked) every affected male will produce a female carrier if mothercarrier: - 50% sons affected - 50% daughters carriers

Answer 62

Duchenne's and Becker's haemophilia A some types of albinism Fragile X syndrome

Answer 63

multi-factorial/polygenic inheritance imprinting and uniparental disomy mitochondrial inheritance

Answer 64

combinaton between the pre-disposing genes and the lived environment spina bifida folic acid deficiency linked to SB and you have a higher risk if you had a sibling with it

Answer 65

for most genes both copies are expressed some genes only are maternal or paternal expressed - IMPRINTING Prader-willi and Angleman's syndrome - cytogenic deletions of same region of C.15q or by uniparental disomy of C.15 (both copies of 15 come from same parent)

Answer 66

Chromosome 15 occurs when failure to inherit the active paternal gene occurs so either inherit 2 copies from mum or an abnormal copy from dad

Answer 67

hypotonia (even from neonate) learning difficulties obesity (due to hyperphagia) small genitalia

Answer 68

Chromosome 15 abnormality failure to inherit active maternal gene 2 copies from dad Or abnormal copy from mum

Answer 69

severe learning difficulty ataxia broad based gait characteristic facial appearance epilepsy 'happy puppet' - unprovoked laughing and clapping microcephalyb

Answer 70

1. confirmation of a clinical diagnosis 2. detect female carriers for X-linked disorders 3. detect carriers in auto rec disorders (siblings of CF patients) 4. pre-symptomatic diagnosis of auto dom disorders (Huntington's disease)

Answer 71

Sorry not an option Not performed on healthy children, wait until old enough until informed consent

Answer 72

1. involved in making diagnoses 2. to explain the diagnosis to the family 3. discuss prognosis 4. discuss options available - genetic testing, screening, prenatal diagnosis 5. refer to appropriate specialists for management

Answer 73

preconception advice for someone with a FHx of a genetic disorder antenatal from paeds - development delayed, dysmorphic features carrier testing for known family history adult onset conditions - advice whether they should have test if parents did post-mortem - unexplained death to look for genetic cause

Answer 74

TORCHS toxoplasmosis other (HIV) Rubella CMV HSV Syphillis

Answer 75

catch from cat poo treatment - spiromycin

Answer 76

encephalitis subacute sclerosing pan-encephalitis febrile convulsions giant cell pneumonia diarrhoea appendicitis myocarditis

Answer 77

degenertive condition of the hip joint due to avascular necrosis of the femoral head more common in boys features - hip pain (may present as knee pain), limp, shortening of affected leg, pain on int and ext rotation of hip imaging - flattening of the femoral head

Answer 78

failure to pass meconium symptoms of bowel obstruction - abdo distension, bilious vomiting failure to thrive, low birthweight, size PR exam = stool ejection more common in boys

Answer 79

rectal biopsy

Answer 80

congential cataracts congenital heart disease sensorineural deafness

Answer 81

MMR vaccine if you see a pregnant lady - check they have MMR vaccine

Answer 82

90% babies normal at birth 5% may develop sensorineural hearing loss later in life

Answer 83

no no vaccine or antiviral therapy for CMV so screening no appropriate

Answer 84

eating infected meat contact with infected cat faeces pregnant women not screened

Answer 85

most infants born asymptomatic other: retinopathy cerebral calcification hydrocephalus long term neuro disability

Answer 86

<20 weeks - risk of skin scarring and neuro damage 2 days before or 5 days after delivery - high viral load (fetus unprotected by maternal ABs) - mortality 30%

Answer 87

varicella zoster immunoglobulin acyclovir

Answer 88

YES So is HIV and hep B

Answer 89

penicillin if fully treated a month or more before delivery - infant does not require treatment and has a good prognosis if any doubt adequate maternal treatment - infant also treated with penicillin

Answer 90

10 normal = between 7 and 10

Answer 91

cardio resp colour reflexes tone

Answer 92

detect congential abnormalities not already identified at birth - heart disease, DDH check for potential problems that could arise due to family history provide an opportunity for parents to ask questions about baby

Answer 93

congenital heart disease development dysplasia of hip (DDH) Talipes (clubfeet) - positional or true T21 cleft lip and palate urogenital - hypospadias, undescended testes spina bifida

Answer 94

structural heart defect patent ductus arteriosus anaemia of prematurity

Answer 95

surfactant deficiency - risk of resp distress syndrome chronic lung disease of prematurity (bronchopullmonary dysplasia) - more prone to pneumonia and viruses - give palivizumab to protect against RSV pneumothorax apnoea, bradycardia and desaturations

Answer 96

necrotising enterocolitis - underdeveloped gut bacteria = swollen gut and pedematous and risk of perforation (red, shiny, tender abdo in babies) - breast milk protective feeding problems / nutrition - slower establishment of feeding, unable to suck and swallow until 33-34 weeks so will need IV - build feeds up slowly to reduce risk of NEC

Answer 97

hypoglycaemia - lack of glycogen stores developed hypocalcaemia - kidneys and parathyroid not fully developed electrolyte and fluid imbalance and hypothermia - excess losses through thin skin osteopenia or prematurity

Answer 98

long term neuro issues apnoea of prematurity - brain stem not fully myelinated until 32-34 weeks so the pontine resp centre is not fully developed intraventricular haemorrhage - diagnose with cranial USS

Answer 99

preterm babies in general more prone to infections Group B strep E.coli and other gram neg fungal infections - candida viral infections - herpes from genital tract during delivery

Answer 100

retinopathy of prematurity occurs due to hyperoxic insult whilst the blood given vessels are still developing (premies baby giving O2 as they are struggling to breathe and end up damaging their eyesight)

Answer 101

<48 hours - infection generally from the birth canal >72 hours - source of infection usually from the environment (catheters for nutrition, tracheal tubes, skin breaking procedures - drawing blood) - most common coagulase negative strep (epidemrins)

Answer 102

prolonged ROM if mother has fever during labour or develops one shortly after birth if infant is pre-term if mother is GBS+ve or has another genital tract infection

Answer 103

being on ICU or NICU - possibility of exposure t patogens and cross contamination

Answer 104

fever/hypothermia/temp instability poor feeding vomiting apnoea and bradycardia resp distress abdo distension jaundice irritabilit seizures lethargy/drowsiness

Answer 105

pneumonia septicaemia occasional meningitis mortality - 10%

Answer 106

pre-term babies prev baby with GBS infection prolonged ROM fever > 38 degree during labour intrapartum ABs given to these high risk ladies if possible

Answer 107

unpasturised milk soft cheese undercooked poultry can cause miscarriage, pre-term delivery and fetal infection

Answer 108

common - clean with water or saline more troublsome discharge - maybe due to staph or strep - neomycin eye ointment gonococcal infection - purulent discharge, 48 hours after birth, gram stain, treat with penicillin or 3rd gen cephalosporin - cleanse frequently chlamydia trachomatis eye infection - purulent, 1-2 weeks of age, identify with immunofluroscent staining, treat with oral erythromycin for 2 weeks

Answer 109

Hep B vaccine shortly after birth to prevent vertical transmission complete vaccine course during infancy and check antibody response

Answer 110

1. CXR 2. urine sample 3. LP - CSF sample 4. blood cultures 5. FBC 6. U&E's

Answer 111

perform a septic screen commence treatment without waitng for culture results

Answer 112

IV broad spec gram +ve and -ve cover BETA LACTAM (amoxicillin) for gram +ve aminoglycoside (gentamycin) for gram -ve if cultures negative and child clinically well after 48 hours then ABx can be stopped

Answer 113

to prevent kernicterus this is billirubin induced encephalopathy caused by unconjugated bilirubin being deposited in the brain this happens because babies blood brain barriers are not well developed so allow billirubin to cross the BBB

Answer 114

physiological jaundice infection haemolysis/haemolytic anaemia metabolic disease

Answer 115

60% of neonates experience it

Answer 116

congenital infection haemolytic disorders: - Rhesus incompatibility - ABO compatibility - G6PD deficiency - spherocytosis - pyruvate deficiency

Answer 117

physiological breast milk jaundice infection - UTI haemolysis - G6PD deficiency, ABO compatibility bruising polycythaemia Crigler-Najjar syndrome

Answer 118

unconjugated: - physiological or breast milk jaundice - infection (UTI) - hypothyroidism - haemolytic anaemia (G6PD def) - high GI obstruction conjugated - bile duct obstruction (biliary atresia) - neonatal hepatitis

Answer 119

no underlying cause behind mild or moderately jaundiced babies bilirubin rises as infant adapts from fetal life - babies RBC have shorter lifespan (70), compared to adult (120), so increased destruction leads to higher amount of circulating bilirubin hepatic bilirubin metabolism is less efficient in the first few days of life can only be called physiologicla after other causes have been considered and ruled out

Answer 120

UTI (investigate - urine sample, blood culture, CXR, FBC)

Answer 121

persisten/prolonged jaundice most jaundice between 2days-3weeks is physiological however, prolonged is more likely to be sinister

Answer 122

looks slightly yellow on skin or eyes often only visible when outside in sunlight

Answer 123

pale stools dark urine probably off feeds and generally unwell in themselves possible itching/scratch marks on their skin

Answer 124

serum bilirubin level transcutaneous billirubin measurement if suspicious biliary atresia - USS liver is gold standard diagnosis

Answer 125

age of onset (prolonged more worrying) severity of jaundice rate of change gestation - pretemr infants more susceptible clinical condition - pyrexia, hypothermia

Answer 126

PHOTOTHERAPY - blue-green (450nm wavelength) - converts unconjugated bilirubin into harmless water-soluble pigment by photodegeneration - no long term sequeale - cover infants eyes - side effects - temp, instability, rash, bronze discolouration EXCHANGE TRANSFUSION - if bilirubin levels are too high to be treated with phototherapy

Answer 127

preterm infants - lack of glycogen stores IUGR infants - lack of glycogen stores babies born to diabetic mothers - have hyperinsulinaemia in response to maternal hyperglycaemia large for dates polycythaemia unwell for any reason

Answer 128

jitteriness irratibility apnoea lethargy drowsiness seizures

Answer 129

regular blood glucose monitoring at bedside prevent by early and frequent milk feeding if needs treating - IVI dextrose via central venous catheter

Answer 130

assess whether a childs overall height is abnormal - below 2nd or above 98th centile ass whether a child is failing to grow to their potential - drop below centile line assess whether a child i losing or gaining weight to quickly - could be a sign of pathology

Answer 131

for boys ((Dad + mum height in cm)/2)+7 for girls ((Dad + mum height in cm)/2)-7

Answer 132

breast development (thelarche) usually occurs between 8.5-12.5 years menarche occurs on average 2.5 years after the start of puberty

Answer 133

testicular enlargement >4ml in volume (measured using a Prader Orchidometer) usually occurs between 10-14 years of age

Answer 134

girls - between 8-12 boys - between 9-13

Answer 135

early in girls <8y/o early in boys <9y/o more worried about early puberty in boys - most commonly a brain tumour (pituitary adenoma) in girls, early puberty more common

Answer 136

girls >13 y/o boys >14y/o more worrying in gilrs - can be sign of abnormal karyotype such as Turner's or primary ovarian failure

Answer 137

height below the 2nd centile

Answer 138

familial - parental shortness, fall in mid-parental range height IUGR and extreme prematurity constitutional delay of growth and pubery endocrine - GH deficiency, steroid excess (Cushing's), hypothyroidism syndromes - Turner, Noonan, Downs Chronic illness and failure to thrive psychosocial deprivation skeletal dysplasias - disproportionate limb and back length

Answer 139

serial measurements on growth chart calculate mid-parental height bone age scan - wrist X-ray FBC and U&E pituitary function test (GH) dexamethasone suppression test (Cushing's) TFT karyotype coeliac antibodies (endomysial and ant-TTG)

Answer 140

height above the 98th centile

Answer 141

familial obesity syndrome - Marfan's hyperthyroidism precocious puberty congenital adrenal hyperplasia excess GH secretion - true gigantism (then after puberty => acromegaly)

Answer 142

breast development before 8 y/o usually idiopathic/familial can occasionally be late presenting CAH

Answer 143

secondary sexual characteristics before 9 y/o uncommon usually brain tumour CAH gonadal tumour

Answer 144

full history + ages parents hit puberty USS of uterus and ovaries boys - MRI brain

Answer 145

boys - treat cause girls - if they okay, leave them. if not GnRH analogues to stop puberty progression until ready

Answer 146

absence of pubertal development by 14 years constituional delay (mum and sisters also did) systemic disease - Crohn's, organ failure, CF hypothalamic causes - stress, anorexia, excessive athletic training

Answer 147

absence of pubertal development by 15 y/o usually familial/constituional - may give testosterone if really bugging them may be due to chronic disease - Crohn's Cf, Coeliac

Answer 148

identify and treat underlying pathology reassure testosterone for boys oestradiol for girls

Answer 149

produced by the sex-determining region of the Y chromosome Role = prevent mullerian duct from persisting and instead encourage development of Wolffian ducts - stops fetus from being females and instead makes them male

Answer 150

a condition where there is reduced production of cortisol (+/- aldosterone) most commonly due to 21 hydroxylase deficiency the cortisol and aldosterone pre-cursors are instead converted into testosterone (which in gilrs causes virillisation of the external genitalia) the reduced amount of circulating cortisol triggers a negative feedback response causing more ACTH to be released from the anterior pituitary this then stimulates the adrenal gland to grow - hence hyperplasia

Answer 151

autosomal recessive

Answer 152

consanguinous parents

Answer 153

femal babies - ambiguous genitalia (clitoral engorgement, fusion of labia) male babies - salt losers - slat-losing adrenal crisis - vomiting, weight loss, floppiness, circulatory collapse non salt-losing male babies - precocious puberty, tall stature, large muscles females may also have preociouc puberty (if they have not already present with ambiguous genitalia)

Answer 154

low plasma sodium high plasma potassium metabolic acidosis hypoglycaemia

Answer 155

IV saline IV dextrose IV hydrocortisone

Answer 156

affected females - corrective surgery for external genitalia internal genitalia not corrected until puberty/sexually active as they will need to use vaginal dilators to keeps passage patent medical treatment - lifelong hydrocortisone (glucocorticoid) and in salt-losers lifelong fludrocortisone (mineralcorticoid) NaCl may also need added before weaning sick day adjustment of cortisol

Answer 157

determine karyotype adrenal and sex hormone levels USS of internal structure and gonads

Answer 158

glucocorticoid = cortisol, control glucose mineralocorticoid = aldosterone, controls salt, which is a mineral

Answer 159

has the optimum macronutrients needed for baby contains maternal ABs to protect against infection free helps mum lose the baby weight 'breast feeding contraception' helps maternal and baby bonding reduced the risk of NEC in preterm infants can help reduce the risk of post-menopausal breast cancers

Answer 160

mum has to do all feeds unless shes expressed milk unknown intake so hard to quantify baby feeding amount breast milk jaundice transmission of drugs/infections from mum to baby can be painful for mum can be upsetting if baby doesn't want to feed insufficient vitamin D and K in breast milk - introduce solids at 6 months (for vit D) and Vit K - injection at birth

Answer 161

failure to gain adequate weight or achieve adequate growth at a normal rate for age OR suboptimal weight gain in infants and toddlers can also be weight/growth faltering serial measurements on growth charts is needed for the diagnosis to be made

Answer 162

mild - fall across 2 centiles lines on growth chart severe = fall across 3

Answer 163

consider overall clinical picture - normal child will have: - no symptoms - happy, alert, responsive, normal milestones - parents also short - may have been extremely premature

Answer 164

MOST CHILDREN failing to thrive will be due to non-organic causes any form of socioeconomic deprivation maternal factors - depression, poor understanding of baby's needs poor housing poverty inadequate social support/lack of extended family neglect/abuse inadequate calorie intake (under-nutrition)

Answer 165

impaired suck/swallow - inadequate intake - cleft palate, neuro-motor dysfunction, CP cardiac disease - breathlessness during feeding malabsorption - Coeliac, CF, food intolerance, short gut syndrome chronic illness - renal, liver, cardiac disease excessive calorie loss - vomiting, protein enteropathy increased caloried requirement - malignancy, throtoxicosis, CF chromosomal abnormalities - Down's

Answer 166

full history - social factor focus Examine - heart murmur, abdo distension, cough bloods - FBC, U&E, CRP/ESRm, Coeliac screen, TFT CXR and swab - CF

Answer 167

increase parental support paediatric dietician SALT specialist for paediatric problem (CF - resp) social services nursery placement hospital admission organic - identify and treat cause

Answer 168

appendicitis uncommon under the age of 3

Answer 169

intussusception between 2 months-2 years usually at ileocaecal valve

Answer 170

acute appendicitis non-specific and mesenteric adenitis intussusception Meckel's malrotation Medical cause - Lower lobe pneumonia, DKA, hepatitis, pyelonephritis Boys - testicular torsion or strangulated hernia

Answer 171

abdo pain - intially central colicky and then localise to RIF anorexia vomiting (normally only a few times)

Answer 172

flushed face with bad breath low grade fever abdo pain aggravted by movement persistent tenderness with guarding at RIF (McBurney's) Rebound tenderness Rosving's signs - LIF pressure = RIF pain

Answer 173

non specific abdo pain = pain resolves in 24-48 hours pain less severe than appendicitis tenderness (and position) variable often accompanied by URTI and cervical lymphadenopathy

Answer 174

paroxsymal, severe, colicky pain child become pale and draws up legs during pain sausage shaped mass on abdo palpation inconsolable crying passage of RED CURRANT JELLY STOOLS abdo distension shock vomiting

Answer 175

severe rectal bleeding - neither bright red or true melena can present as intussusception can present as volvulus (bowel obsturction) can present as diverticulitits which mimics appendicitis

Answer 176

usually presents in first 1-3 days of life with intestinal obstruction may present at any age with volvulus causing obsruction and ischaemic bowel clinical features: - bile or blood stained vomit - abdo pain - tenderness - from peritonitis or ischaemic bowel often associated with exopthalmous and congenital diaphragmatic hernia

Answer 177

laparotomy or laparoscopy showing normal appendix and large mesenteric lymph nodes

Answer 178

USS - target sign others - AXR - distended small bowel and no gas distally

Answer 179

urgent upper GI contrast study need to do whenever there is blood or bile stain vomiting

Answer 180

appendicetomy

Answer 181

leave alone in some - appendicetomy may be done is symptoms persist and mesenteric adenitis can be definitley diagnosed

Answer 182

aggressive fluid resus reduction via air enema - USS guidance laparotomy if above does not work

Answer 183

fluid resus surgical resection

Answer 184

urgent surgical correction

Answer 185

recurrent pain, sufficient to interrupt normal activities lasting for at least 3 months

Answer 186

GI - IBS, constipation, non-ulcer dyspepsia, abdo migraine, gastritis, PUD, IBD, malrotation, coeliacs gynae - dysmenorrhoea, ovarian cysts, PID, pregnancy liver - hepatitis pancreatitis UTI psychological - bullying, abuse, stress

Answer 187

change of bowel habit sensation of intra-abdo events (bloating) pain - often worse before and relieved by defecation mucousy stools bloating feeling incomplete defecation constipation/diarrhoea/mixed

Answer 188

epigastric pain postprandial vomiting belching bloating early satiety heartburn

Answer 189

(classic cranial migraines associated with abdo pain along with headaches) pain is: - midline - paroxysmal - associated with facial pallor family history of migraine

Answer 190

epigastric pain at night - haematemesis - duodenal ulcer diarrhoea, weight loss, growth failure, blood in stools - IBD vomiting - pancreatitis dysuria, secondary enuresis - UTI Vomiting and abdo distension - malrotation

Answer 191

Guided by clinical features Essential to do urine MC&S as UTI can cause abdo pain Endoscopy if dyspeptic symptoms Colonoscopy if any rectal bleeding or lower abdominal symptoms IBS – diagnosis of exclusion

Answer 192

Can talk to a dietician about possible exclusion diets to see if any particular food aggrevates symptoms Manage stress Antispasmodics (for the pain) – i.e. mebeverine Anti-diarrhoea – loperamide Laxatives if they are suffering constipation

Answer 193

If H. pylori - needs eradicating with triple therapy

Answer 194

Pizotifen – a serotonin receptor antagonist Used as prophylaxis in children with frequent, severe symptoms

Answer 195

Non-forceful return of small amounts of milk Usually accompanied by wind Occurs normally in nearly all babies

Answer 196

Non-forceful return of milk Larger, more frequent losses than possetting Usually indicates gastro-oesophageal reflux

Answer 197

Forceful ejection of gastric contents

Answer 198

Reflux (very common) Feeding problems Infection (gastroenteritis, whooping cought, UTI, meningitis) Dietary protein intolerances Intestinal obstruction (pyloric stenosis, atresia, Hirshprung’s, malrotation, intussusception etc.) Inborn errors of metabolism CAH Renal failure

Answer 199

Gastroenteritis Other infections (kidneys, septicaemia, meningitis) Appendicitis Intestinal obstruction (intussusception, maloration, volvulus, adhesions) Raised ICP Coeliac disease Renal failure Inborn errors of metabolism Torsion of testis

Answer 200

Gastroenteritis Other infection – kidneys, septicaemia, meningitis Peptic ulcers Appendicitis Migraine Raised ICP Coeliac DKA Alcohol/drugs Cyclical vomiting syndrome Bulimia Pregnancy Testicular torsion

Answer 201

Bile stained – exclude intestinal obstruction Blood in the vomit – oesophagitis, peptic ulcer, oral/nasal bleeding, malrotation Projectile vomiting in the first few weeks of life – exclude pyloric stenosis Associated symptoms – CNS, GI, UT infections Vomiting after paroxysmal cough – whooping cough (Pertussis) Dehydration or shock – could be an obstruction – 3rd spacing 🡪 shock Abdominal distention – exclude lower intestinal obstruction and strangulated inguinal hernia

Answer 202

VITAL SIGNS ASSESS FOR DEHYDRATION AND SHOCK Rehydrate and support Find and treat underlying cause

Answer 203

Faltering growth/failure to thrive Oesophagtis +/- stricture Apnoea Aspiratation, wheezing, hoarseness – often comes as referrals from respiratory IDA Seizure like events/opsthotonos (acid causes arching of the back to adjust position)

Answer 204

Barium swallow and meal Endoscopy PPI trial

Answer 205

Position of their feeds - on their left, hold them upright Thicken feeds - gaviscon Drugs - antacid, H2 blocker (ranitidine), PPI Surgery – fundoplication

Answer 206

Change in the consistency of stools (loose/liquid) And/or increase in the frequency of passing stools Acute – less than 2 weeks

Answer 207

Viruses – rotavirus, norovisu, enterovirus, many more Bacteria – C.diff, E.coli, salmonella Parasites – giardia Other infections – otitis media, tonsillitis Food allergies and other diseases

Answer 208

Complication of diarrhoea Happens because the water loss exceeds the sodium loss Difficult to detect – blood test shows serum sodium > 145 Unusual and serious Irritable with doughy skin Water shifts from intracellular to extracellular Rehydration should be slow

Answer 209

Jittery movements Increased muscle tone Hyperreflexia Convulsions Drowsiness/coma Doughy skin Irritable

Answer 210

Firstly – ASSESS FOR DEHYDRATION – cap refill, pinch test, sunken eyes, mucous membranes Look for an underlying cause

Answer 211

Encourage normal diet Assess and treat dehydration – either oral or IV/NG if severe Supportive therapy Treat underlying cause if possible – antibiotics, probiotics

Answer 212

Cow’s milk protein allergy This is NON IgE mediated

Answer 213

Loose stool Vomiting Failure to thrive Reflux Mouth ulcers Atopic history (eczema, asthma, hayfever) Bloody stools Constipation Very rarely – anaphylaxis

Answer 214

Explosive watery stools Abdominal distention Flatulence Audible bowel sounds

Answer 215

Elimination damage

Answer 216

Change feeds – hydrolysed or powdered milk feeds Hope that the child will “grow out of it” eventually

Answer 217

Small intestine disease – coeliac Exocrine pancreatic dysfunction – i.e. in CF (absence of lipase, proteases and amylase lead to defective digestion) Cholestatic liver disease/biliary atresia – malabsorption of fat due to lack of bile salts Short bowel syndrome – resection due to congenital abnormalities/NEC. Nutrient, water and electrolyte malabsorption Loss of terminal ileum function – resection, Crohn’s. absent bile acid and vitamin B12 absorption

Answer 218

Abnormal stools – difficult to flush, extremely offensive odour Failure to thrive/poor growth Specific nutrient deficiencies e.g. iron deficiency anaemia, B12 deficiency

Answer 219

Present in the first few years of life after introduction of gluten in cereals Generally irritable Abnormal stools (smelly, diarrhoea, floating) Abdominal distention Buttock wasting Failure to thrive/poor growth

Answer 220

Antibodies – endomysial antibodies and tissue transglutaminase antibodies Gold standard diagnosis – intestinal (jejunal) biopsy showing villous atrophy and crypt hyperplasia

Answer 221

Lifelong gluten free diet

Answer 222

Constipation = painful passage of hard, infrequent stools Encopresis = involuntary soiling

Answer 223

Child may avoid defecating due to pain (i.e. they have a transient superficial anal fissue) Occasionally follows – forceful potty training, use of uncomfortable toilets (on holiday/at school) or psychological family stress

Answer 224

Child avoids toileting due to pain Prolonged faecal status = resorption of fluids = increase in size and constistency Can lead to stretching of the rectum (stools withheld for a long time due to not wanting to defecate) This leads to reduced sensation Which can lead to overflow and soiling

Answer 225

Delayed passage of meconium – Hirshprung’s, CF Fever, vomiting, bloody diarrhoea Failure to thrive Tight empty rectum and palpable abdo mass – Hirshprung’s Ejection of stools on PR – Hirshprung’s Abnormal neuro examination – abdo exam in children should include SPINAL EXAMINATION due to close proximity of the rectum

Answer 226

Acquired megacolon Anal fissues Overflow incontinence (soiling) Behavioural problems

Answer 227

Hirschsprung’s Anorectal malformations Spina bifida Neuromuscular disease Hypothyroidism Hypercalcaemia Coeliac Food allergy/intolerance CF Sexual abuse Poor diet

Answer 228

Clinical diagnosis Abdo exam – hard stools palpable Further Ix only if organic cause suspected

Answer 229

Diet, fluids, exercise Toilet training – sit after breakfast/lunch/dinner Star charts – simple reward scheme Medications Softener – lactulose Movicol Stimulant – senna, dulcolax Bulking agent – fybogel

Answer 230

Affects the mouth to anus Transmural inflammation Discontinuous with skip lesions GRANULOMATOUS Rectal sparing Fissures, fistulae, abscesses and strictures Perianal disease

Answer 231

FAILURE TO THRIVE – poor growth, delayed puberty Abdominal pain Diarrhoea Rectal bleeding Tiredness/malaise Extra GI symptoms – fever, arthritis, uveitis Can present identical to anorexia nervosa – important to think about this

Answer 232

Ulcerative colitis

Answer 233

Clinical examination Endoscopy Histology – look for granulomas Biochemistry – inflammatory markers

Answer 234

Exclusive enteral nutrition – feed them loads of milk for 7-8 weeks and they get better Corticosteroids – prednisolone, budenoside Aminosalicylates – sulfasalazine, mesalazine Antibiotics Immunomodulators – MTX, azathioprine, 6-mecaptopurine Biologics – infliximab, adalimumab Surgery

Answer 235

Affects ONLY the colon And ONLY the mucosa No granulomas No skip lesions – the whole colon is affected Ulcers Increased risk of adenocarcinoma in adulthood

Answer 236

Failure to thrive/weight loss Rectal bleeding Diarrhoea Colicky pain Extra GI – mouth ulcers, arthritis, erythema nodosum, spondylitis

Answer 237

Crohn’s disease Infective causes of colitis e.g. C. Diff

Answer 238

Colonoscopy – friable membranes (bleeding), visible ulcers

Answer 239

Mild attacks – topical steroids when confined to rectum/sigmoid colon, sulfasalazine for more extensive disease Severe disease –systemic steroids 1/3 patients – colectomy (curative)

Answer 240

Very low dose inhaled corticosteroid Plus salbutamol PRN

Answer 241

Used to assess if a patient who has not yet reached age is competent to consent to treatment. Criteria: Understands the professional’s advice Cannot be persuaded to inform their parents Likely to begin or continue sexual intercourse without contraception Physical and mental health will suffer if they don’t get the contraceptive treatment The young person’s best interests require them to receive contraceptive treatment with or without parental consent

Answer 242

Prematurity

Answer 243

Caesarean section

Answer 244

SpO2 < 92% PEF 33-50% Too breathless to talk or feed Heart rate - >125 (>5 years), >140 (1-5 years) Resp. rate > 30/min (> 5 years), > 40 (1-5 years) Use of accessory neck muscles

Answer 245

SpO2 < 92% PEF < 33% Silent chest Poor respiratory effort Agitation Altered consciousness Cyanosis

Answer 246

Infection with the measles virus Droplet infection is the method of transmission Highly contagious

Answer 247

Avoidance of the MMR vaccination

Answer 248

PRODROMAL SYMPTOMS - CCCK: Cough Coryza (runny nose/cold like symptoms) Conjunctivitis Koplick spots (white spots on the buccal mucosa – pathognomonic) RASH – maculopapular, spreads downwards from behind the ears to the whole of the body Fever Marked malaise

Answer 249

Respiratory – pneumonia, otitis media, tracheitis Neurological – febrile convulsions, encephalitis Diarrhoea Hepatitis Corneal ulceratoin Appendicits Myocarditis

Answer 250

Clinical diagnosis Can confirm with serological testing of blood or saliva

Answer 251

VIRAL ILLNESS so supportive treatment only (symptomatic, fluids If needed, isolate if in hospital) Antivirals in immune compromised School exclusion for 5 days from onset of rash Best treatment – preventative vaccine

Answer 252

Unvaccinated children

Answer 253

Fever Malaise Parotitis (swollen parotid gland) May complain of pain eating and drinking May complain of earache Hearing loss can occur – usually unilateral and transient

Answer 254

Viral meningitis and encephalitis Orchitis – reduced sperm count in males Oophoritis Mastitis Arthritis

Answer 255

Clinical diagnosis Management – symptomatic (viral illness) School exclusion for 7 days from onset of parotitis

Answer 256

Winter and spring months (could be a hint in questions)

Answer 257

Maculopapular rash starting on the face and spreading across the entire body NOT itchy in children (but is in adults)

Answer 258

When there is a risk of exposure to non-immune pregnant lady Congenital Rubella – cataracts and sensorineural deafness

Answer 259

No effective anti-viral treatment Usually resolves in 3-5 days Prevention lies in immunisation

Answer 260

Through mucous membranes of skin Kissing Genital contact Vertical transmission at birth

Answer 261

Gingiostomatitis – sores on the lips, gums, tongue and hard palate – can lead to ulceration and bleeding, common reason why child may refuse to eat or drink Cold sores Herpetic whitlows – in thumb suckers Conjunctivitis and inflammation of the eyelids CNS infection – aseptic meningitis (self-resolving), encephalitis

Answer 262

Clinical diagnosis Acyclovir Give during pregnancy especially to stop transmission to baby

Answer 263

Varicella zoster virus (human herpes virus 3) May also cause shingles virus later in life – reactivation and then a rash appears in a dermatomal distribution

Answer 264

Droplet infection via the respiratory route Highly infectious – contagious from 2 days before the rash appears and until it disappears

Answer 265

Vesicular rash Very itchy Starts on the head and trunk and progresses to the peripheries

Answer 266

High fever – 38-39*C Usually stops when the rash appears

Answer 267

Haemorrhagic lesions Pneumonitis Progressive and widespread infection DIC

Answer 268

Camomile lotion to stop the itching Keep away from high risk individuals – i.e. don’t put them anywhere near a clinical area in which there are immune suppressed patients

Answer 269

Human varicella zoster immunoglobulin (HVZIG) (bone marrow transplant patients, congenital or aquired immune deficiency, steroids, neonates)

Answer 270

Fever Malaise and extreme lethargy Tonsillopharyngitis Prominent cervical lymphadenopathy Petechiae on soft palate Splenomegaly Hepatomegaly Maculopapular rash Jaundice Caused by Epstein Barr virus

Answer 271

Positive monospot test Seroconversion – IgG to Epstein-Barr virus antigens Treatment = symptomatic

Answer 272

Roseola infantum Generalised macular rash which appears after the prodromal fever wanes Common cause of febrile convulsions

Answer 273

Most common during spring Fever, malaise, headache, myalgia – prodromal Characteristic cheek rash – looks like they have been slapped on the cheeks Progresses to maculopapular rash on the trunk and limbs Caused by parvovirus b19

Answer 274

Infects the red cell precursors in the bone marrow Can lead to aplastic crisis in children with chronic haemolytic anaemias (sickle cell, thalassaemia) and immunocompromised children

Answer 275

Hand, foot and mouth disease

Answer 276

Painful vesicular lesions on the hands and feet Painful ulcers in the mouth and on the tongue Systemic features are mild Subsides in a few days

Answer 277

Direct effect – abscess, cellulitis, oesteomyeltitis, impetigo, septic arthritis, septicaemia Toxin-mediated (indirect) – TSS, food poisoning Toxin-mediated (direct) – scalded skin syndrome

Answer 278

Strep. Pyogenes (group A beta haemolytic strep) Same bacteria that causes strep throat

Answer 279

Honey coloured crusted lesions, often on the face Staph aureus or group A strep (pyogenes) Treatment – fucidic acid (topical treatment) or erythromycin

Answer 280

Staph aureus

Answer 281

Droplet infection Strep. Pneumoniae can be carried asymptomatically in the nasopharynx of health children – only spread and cause disease when they cough or sneeze

Answer 282

Pharyngitis Otitis meida Conjunctivitis Invasive disease – pneumonia, sepsis, meningitis

Answer 283

Toxin producing staph and strep High fever Diffuse macular rash Hypotension Shock Other symptoms – muscle pain, vomiting, headache, altered consciousness, desquamation of the hands and feet

Answer 284

ICU to manage shock Surgical debridement of infected areas IVIG – neutralises circulating toxin

Answer 285

Fever malaise Purulent, crusting, localised infection (around eyes, nose and mouth) Widespread erythema and tenderness of the skin Cause – staphyloccocus aureus toxin Treatment – IV fluclox, monitor fluid balance, analgesia

Answer 286

Large visible necrotic areas of skin – affecting all the layers of the skin and fascia Usually staph or group A strep (pyogenes) Treatment – IV abx PLUS surgical debridement

Answer 287

6 months to 5 years More common in japanese and Afro-Caribbean infants

Answer 288

Unknown Thought to be related to bacterial toxins

Answer 289

Fever for > 5 days Plus 4/5 of the following: Cervical lymphadenopathy Bilateral conjunctival injection Changes to the hands and feet – peeling of the skin on the hands and feet/palmar and plantar erythema A rash Changes the mouth or throat – dry, cracked lips/red swollen tongue

Answer 290

Coronary artery aneursysms Need to perform a echocardiogram (the diagnosis is otherwise clinical so no other investigations need to be performed)

Answer 291

IVIG Aspirin

Answer 292

Due to the risk of Reye’s syndrome (causes swelling of the liver and brain)

Answer 293

Meningococcal infection (Neisseria meningitidis) Which may or may not be accompanied by meningitis !!

Answer 294

Group B streptoccous Or gram negative organisms acquired from the birth canal

Answer 295

Sickle cell disease Immunodeficiency

Answer 296

Fever Poor feeding Miserable/irritable Lethargy/drowsiness History of focal infection – meningitis, osteomyelitis, gastroenteritis, cellulitis

Answer 297

Fever Purpuric non-blanching rash (meningococcal septicaemia specifically) Irritability Signs of shock Cold peripheries Reduced cap refil Pale Hypotension Tachycardia Oliguria

Answer 298

SEPTIC SCREEN: FBC, U&E Blood cultures Urine sample – MC&S CSF sample Chest XR Acute phase reactant e.g. CRP

Answer 299

Aggressive fluid resuscitation Potential transfer to intensive care Start broad spectrum antibiotics before cultures come back

Answer 300

Sterile meningitis can be caused by: Malignancy Autoimmune diseases

Answer 301

Neonates - GBS, E.coli, listeria monocytogenes 1 months – 6 years – meningococcus, pneumonoccus, haemophilus influenzae >6 years – meningococcus and pneumococcus Rarely - TB

Answer 302

Enteroviruses EBV Adenoviruses Mumps

Answer 303

Fever Headache Photophobia Lethargy Poor feeding/vomiting Irritability Hypotonia (the baby or child will be floppy) Drowsiness Loss of consciousness Seizures

Answer 304

Fever Purpuric non-blanching rash (if meningococcal sepsis) Neck stiffness Positive Brudzinki’s sign – flexion of the neck causes flexion of the knees Positive Kernig’s sign – back pain on extension of the knee Bulging fontanelle Opisthotonos

Answer 305

FBC and white cell Blood glucose and gas CRP U&E LFTs Cutures - blood, throat swab Lumbar puncture (unless contraindicated) Consider CT/MRI brain

Answer 306

Signs of raised ICP Focal neurological signs Bleeding problems Local infection at site of LP Cardiorespiratory instability If it will unduly delay the starting of antibiotics

Answer 307

Hearing loss Long term neurological impairment Hydrocephalus Cerebral abscess Encephalitis

Answer 308

Cloudy colour Raised polymorphs (neutrophils, eosionophils, basophils) Raised protein Decreased glucose

Answer 309

Clear colour CSF Raised lymphocytes Normal protein Normal glucose

Answer 310

Immediate IV antibiotic treatment – cefotaxime or ceftriaxone Supportive therapy (IV fluids, pain relief)

Answer 311

Meningitis B – 8 weeks, 16 weeks, 1 year Meningitis C – 1 year ACWY – offered to teenagers/first year university students

Answer 312

Rifampicin

Answer 313

Inflammation of the brain parenchyma Can be difficult to differentiate from meningitis clinically at first

Answer 314

Enteroviruses Respiratory viruses Herpes viruses

Answer 315

Fever Headache Photophobia Neck stiffness (same clinical presentation as meningitis) Can also feature behavioural change

Answer 316

FBC U&E Lumbar puncture Blood cultures

Answer 317

Antibiotic cover in the event it could be bacterial meningitis Acyclovir - to cover HSV possibility

Answer 318

Mantoux test Quadruple therapy – RIPE Rifampicin Isoniazid Pyrazinamide Ethambutol 6 months of R + I, 2 months of P + E (6, 6, 2, 2)

Answer 319

Vertical transmission from an HIV infected mother

Answer 320

Symptoms of immunosuppression: Recurrent bacterial infections Candidiasis Opportunistic infections e.g. Pneumocystis jiroveci Severe failure to thrive Malignancy Any usual collections of infections should make you think of immune deficiency

Answer 321

HIV DNA CPR blood test

Answer 322

Avoid breast feeding Use of antenatal, perinatal and post-nastal antiretroviral to suppress viral replication Avoidance of labour and contact with the birth canal – planned C-section

Answer 323

Induce T and B cell (antibody) immunity for organisms/toxins Induce immunological memory – so if you come into contact with the organism again, you can fight it due to the presence of memory immunity Herd immunity - important as well as individual protection as it protects those who haven’t been immunised

Answer 324

Active immunity – give them part of the pathogen Passive immunity – give them antibodies to the pathogen instead

Answer 325

MMR BCG Nasal flu Rotavirus Chickenpox

Answer 326

Tetanus Diphtheria Whooping cough Polio Measles Mumps Rubella Hib (Haemophilus influenza B) Men C Prevnar 13 (pneumococcal – strep pneumoniae) Rotavirus Meningitis B

Answer 327

Pneumococcus Meningococcus ACWY Varicella TB Hepatitis Influenza HPV

Answer 328

Tetanus Diptheria Pertussis Polio HiB Prev 13 Men B

Answer 329

Tetanus Diptheria Pertussis Polio Hib Men C

Answer 330

Tetanus Diptheria Pertussis Polio Hib Prevnar 13 (pneumococcal) Men B (same as at 2 months – boosters of all of these)

Answer 331

Hib Prev 13 Men B Men C MMR

Answer 332

Tetanus Dip Pertussis Polio Flu

Answer 333

Tetanus booster Men ACWY HPV (girls only) Flu (some)

Answer 334

A hypersensitive reaction initiated by specific immunoglobulins Can be IgE mediated – i.e. peanut allergy Or non-IgE mediated – i.e. coeliac disease

Answer 335

Objectively reproduceable symptoms or signs following a defined stimulus (e.g. food, drug, venom) at a dose tolerated by a normal person

Answer 336

A personal/familial tendency to produce IgE in response to ordinary exposures to allergens (results in ashtma, hayfever, conjunctivitis or eczema)

Answer 337

A severe, life threatening hypersensitivity reaction

Answer 338

Stat does of adrenaline (i.e. giving through an epi pen)

Answer 339

Coryza Conjunctivitis Postnasal drip causing cough

Answer 340

Antihistamines (used singly or in combination) Steroid nasal sprays Cromoglicate eye drops Leukotriene inhibitors Oral steroids Specific immunotherapy

Answer 341

Severe combined immunodeficiecny X-Linked agammaglobulinaemia (assoc. w pneumocystis jiroveci) Defects of bacterial phagocyte function Defects in leucocyte function Minor immunoglobulin abnormalities Opsonisation defects

Answer 342

Malignancy HIV Bone marrow transplant Steroid/immune supressant drugs

Answer 343

Candida PCP (pneumocystis jiroveci) Aspergillus These are all fungal infections

Answer 344

Full blood count White cell count Lymphocytes Blood film Neutrophils complement Immunoglobulins

Answer 345

Antibiotic prophylaxis to prevent infection – e.g. co-trimoxazole to prevent PCP Appropriate ABx to treat infections Immunoglobulin replacement therapy for defects in antibody function or production Bone marrow transplant for severe immune deficiency Hope for the future – gene therapy (due to the genetic component of these illnesses)

Answer 346

6-8 Mostly self limiting – ear, nose, throat (URT) Some are more dangerous – bronchiolitis, pneumonia (LRT)

Answer 347

Nasal discharge and blockage Fever Painful throat Earache Cough may also be present and troublesome

Answer 348

Features – clear or mucopurulent nasal discharge, nasal blockage Commonest pathogens are VIRAL – rhinoviruses (hundreds of subtypes), coronaviruses, RSV

Answer 349

Education to parents that colds are self-limiting and have no specific curative treatment (to reduce anxiety and reduce un-necessary visits to the doctor) Paracetamol or ibuprofen – for fever and pain NO BENEFIT OF ANTIBIOTICS as it’s a viral infection and secondary bacterial infections are very uncommon

Answer 350

Usually viral – adenoviruses, enteroviruses, rhinoviruses In older children - group A beta haemolytic strep (pyogenes) is very common Leave alone if viral Fluclox if suspect strep throat

Answer 351

Tonsillitis = inflammation of the pharyngeal tonsils Commonest organisms = group A beta-haemolytic strep and EBV Viruses are the most common cause, however it’s clinically impossible to distinguish between bacterial and viral

Answer 352

Headache Apathy Abdominal pain Tonsillar exudate Cervical lymphadenopathy

Answer 353

Penicillin Erythromycin if allergic Many cases treated with Abx even when only 1/3 estimated to be bacterial Amoxicillin avoided as can cause rash if due to EBV

Answer 354

>7 infections of recurrent acute tonsillitis in a year Obstructive sleep apnoea/sleep disordered breathing (this is now the main reason for tonsillectomy) A peri-tonsillar abscess (quinsy)

Answer 355

Otitis media with effusion with hearing loss Obstructive sleep apnoea

Answer 356

Presentation: Irritable/miserable child pulling at their ear (ear pain) Fever Most common at 6-12 months of age Most children will experience it at least once

Answer 357

Bright red Bulging appearance Loss of normal light reflection

Answer 358

RSV Rhinovirus Pneumococcus (strep pneumoniae) H. influenzae Moraxella catarrhalis Strep. Pyogenes (group A beta haemolytic)

Answer 359

Ibuprofen and paracetamol for pain 80% resolve spontaneously Amoxicillin

Answer 360

Extracranial – mastoiditis, tympanic membrane perforation, glue ear Intracranial – meningitis, abscess, venous sinus thrombosis

Answer 361

Aka glue ear Recurrent OM infections leading to reducing hearing The most common cause of conductive hearing loss in children

Answer 362

TM appears dull and retracted Often with a visible fluid level

Answer 363

Most cases resolve spontaneously But can be treated with the insertion of grommets (ventilation tubes) – drains the excess fluid away from the tympanic membrane

Answer 364

Infection of the paranasal sinuses Usually viral so symptomatic treatment If bacterial – antibiotics and analgesia

Answer 365

Upper airway obstruction secondary to adeno-tonsillar hypertrophy

Answer 366

Loud snoring Apnoea for 30-45 seconds Disturbed sleep Some children may be obese, underweight, hyperactive in the day or excessively sleepy during the day

Answer 367

Overnight sleep studies Showing intermittent hypoxia and hypercapnia

Answer 368

Adeno-tonsillectomy

Answer 369

Inflammation of the larynx and trachea in children Associated with infection Causes breathing difficulties

Answer 370

Parainfluenza viruses

Answer 371

6 months – 6 years Most common in autumn

Answer 372

Initially – fever and coryza Barking cough – “seal like” Harsh stridor Hoarseness Symptoms often start and are worse at night

Answer 373

FIRST LINE = ORAL DEXAMETHASONE single dose Nebulised epinephrine for severe croup Oral prednisolone Nebulised steroids If < 12 months – low threshold for hospital admission

Answer 374

Haemophilus influenza B (bacterial infection) Life threatening emergency

Answer 375

Due to the introduction of HiB vaccine

Answer 376

Very acute onset High fever in an unwell, toxic looking child Extremely painful throat – cannot speak or swallow – drooling saliva down the chin SOFT stridor Increasing respiratory difficulty Child sits immobile, upright with an OPEN MOUTH to optimise airway No/minimal cough

Answer 377

URGENT hospital admission DO NOT EXAMINE THE THROAT Senior anaesthetist Paediatrician ENT surgeon Intubate/emergency tracheostomy if needed Once airway is secured – blood cultures Start cefuroxime Abx for 3-5 days Rifampicin prophylaxis offered to household contacts

Answer 378

Whooping cough is caused by Bordetella Pertussis (bacterial) It’s a highly infectious form of bronchitis (inflammation of the bronchi)

Answer 379

Coryza for 1 week (catarrhal phase) Then paroxysmal spasmodic cough Followed by classic inspiratory whoop (paroxysmal phase) Spasms of cough often worse at night May vomit after the coughing episodes No breathing difficulty as no gas exchange happens at the bronchi. Whereas bronchiolitis will have severe breathing difficulty

Answer 380

Nasal swab to detect Bordetella pertussis Marked lymphocytosis on blood film

Answer 381

Admit to hospital if suffering cyanotic attacks Erythromycin – treatment for patient and prophylaxis for families Offer vaccine to prevent getting infection again Pregnant women should be vaccinated

Answer 382

Inflammation and infection of the bronchioles Most common respiratory infection of infancy OCCURS IN INFANTS UP TO 1 YEAR OLD RARE AFTER 1 YEAR

Answer 383

Respiratory syncytial virus (RSV)

Answer 384

Premature babies Infants with congenital heart disease Infants with CF Infants with any type of immune deficiency

Answer 385

Coryzal symptoms Dry cough BREATHLESS Wheeze Feeding difficulty – associated with dyspnoea

Answer 386

Tachypnoea Subcostal and intercostal recessions Hyperinflation of the chest Fine end-inspiratory crackles High-pitched wheezes Tachycardia Low O2 SATS Cyanosis/pallor

Answer 387

RSV can be identified on nasopharyngeal secretions using immunofluorescence CXR – hyperinflation of the lungs due to small airways obstruction Blood gas (in severe cases) – lowered arterial oxygen and raised CO2

Answer 388

SUPPORTIVE TREATMENT ONLY due to viral infection Humidified oxygen Fluids if needed – IV, NG Mechanical ventilation in 2% of infants admitted

Answer 389

Palivizumab – a monoclonal antibody vaccine to RSV Given to high risk individuals – preterm, CF, congenital heart/lung disease IM injection once a month

Answer 390

Pneumonia = infection and inflammation of the lung parenchyma Can be viral or bacterial

Answer 391

Group B streptococcus Gram negative enterococci These are caught from the mother’s genital tract

Answer 392

Bacterial – strep pnuemoniae, H. Influenzae, bordetella pertussis, chlamidya, staph aureus Viral – RSV mainly. Also rhino and other respiratory viruses

Answer 393

Strep pneumoniae Chlamidya pneumoniae Klebsiella pneumoniae Myoplasma pneumoniae Mycobacterium TB - should be considered at all ages

Answer 394

Pneumocystis jiroveci TB

Answer 395

Congestion Red hepatisation Grey hepatisation Resolution

Answer 396

Usually preceded by URTI Fever >38.5* Difficulty breathing Cough – productive Lethargy Poor feeding Unwell Chest, neck or abdominal pain Grunting

Answer 397

Tachypnoea Nasal flaring Chest in drawing Chest hyperinflation and wheeze – viral or mycoplasma infection End-inspiratory crackles over the affected area Possible decreased oxygen saturations

Answer 398

Chest X-Ray – dense white area if lobar pneumonia caused by strep pneumoniae Nasopharyngeal aspirate FBC, WCC etc.

Answer 399

O2 sats < 93% Severe tachypnoea and difficulty breathing Grunting Apnoea Not feeding Family unable to provide appropriate care

Answer 400

Supportive – analgesia, O2, fluids Antibiotics - Newborns = broad spec, Older infants = oral amoxicillin or erythromycin

Answer 401

An expiratory, polyphonic breathing sound Created by air being forced through a narrow air passage

Answer 402

Transient early wheezing Non-atopic wheezing in the pre-school child (viral episodic wheeze) IgE-mediated wheezing (atopic ashtma)

Answer 403

Transient early wheezing Viral episodic wheeze Asthma Chronic aspiration of feeds CF Inhaled foreign body

Answer 404

Eosinophils Lymphocytes Mast cells Neutrophils

Answer 405

Virus associated Decreased lung function from birth – small airway calibre Risk factors – maternal smoking, prematurity. NO relation to FH of asthma More common in males Usually “grow out of it” by age of 5

Answer 406

Normal lung function in early life Then become episodic viral wheezers in first 10 years of life Symptoms improve during adolescence No interval symptoms – ONLY GET WHEEZY WHEN THEY HAVE A VIRUS No atopy No benefit from regular ICS Most common cause = rhinovirus (common cold)

Answer 407

PRN salbutamol inhaler

Answer 408

Normal lung function at birth Recurrent wheeze develops with allergic sensitisation (increased IgE in blood, positive skin prick test to common allergens) PERSISTENT symptoms – but may be worse during an infection Risk factors – family history, eczema Symptoms: Cough Wheeze Breathlessness Tight chest Diurnal variation of symptoms

Answer 409

Clinical diagnosis – consider disease pattern (i.e. symptoms only when they have a cold = episodic viral wheezer, symptoms all the time = asthma) Skin prick test for common allergens Peak flow diary Bronchodilator responsiveness – PEF will increase by 1—15% after inhaling a bronchodilator

Answer 410

British thoracic society says that everyone with a new diagnosis of asthma should be given: A SABA (short acting B2 agonist such as salbutamol) AND a low-dose inhaled corticosteroid (such as beclametasone)

Answer 411

In children under 5 – add a leukotrine receptor antagonist (e.g. montelukast) In children 5 or over – add a LABA (long acting beta 2 agonist – such as salmeterol)

Answer 412

Oxygen Nebulised or inhaled beta 2 agonist IV steroid (prednisolone or hydrocortisone) Atrovent nebuliser IV salbutamol bolus Magnesium sulphate/aminophylline/salbutamol infusion Follow this stepwise management

Answer 413

Using a spacer

Answer 414

Adrenal suppression Growth suppression Osteoporosis Diabetes Cataracts Important to ensure your patient is on the minimum possible dose to minimise the side effects

Answer 415

ABCDE of failure to respond: A – Adherence (most common reason is they aren’t taking it properly) B – Bad disease (dose not adequate for severity of disease) C – Choice of drug/device (certain people respond differently to different treatments) D – Diagnosis may be incorrect E – Environment (there’s an obvious environmental trigger making the symptoms worse – e.g. asking about pets and parental smoking in children with asthma)

Answer 416

Recurrent colds Allergic rhinitis (post-nasal drip can cause a cough) Infections – pertussis, RSV, TB Reflux – aspiration can cause cough Cigarette smoking (active or passive) Suppurative lung disease (CF, bronchiectasis, ciliary dyskinesia) TB – MUST be excluded in all children with recurrent cough Habit cough

Answer 417

CF Primary ciliary dyskinesia Chronic aspiration Immunodeficiency TB

Answer 418

Cystic fibrosis transmembrane regulator gene (CFTR) On chromosome 7 Inherited by autosomal recessive inheritence Mutation = Delta F 508 mutation

Answer 419

Diagnosed through the new born screening blood spot programme

Answer 420

Meconium ileus in newborn period Prolonged neonatal jaundice Recurrent chest infections Most commonly = Failure to thrive and Malabsorption, steatorrhoea (foul smelling and mucusy)

Answer 421

Chronic cough Bronchiectasis Rectal prolapse Nasal polyp Sinusitis

Answer 422

Allergic bronchopulmonary aspergillosis (ABPA) Diabetes mellitus (often not insulin-depedent) Cirrhosis and portal hypertension Distal intestinal obstruction (DIOS – equivalent to meconium ileus) Pneumothorax or recurrent haemoptysis Sterility in males Increasing psychological problems

Answer 423

Staph aureus Haemophilus influenzae Pseudomonas aeruginosa

Answer 424

Hyperinflation of the chest due to air trapping Coarse inspiratory crepitations And/or expiratory wheeze Finger clubbing with established disease

Answer 425

95% of patients with CF will die of respiratory failure

Answer 426

Pancreatic exocrine insufficiency – insufficient amounts of lipase, amylase and proteases produced by the pancreas Therefore they cannot digest and absorb food adequately 🡪 failure to thrive, steatorrhoea LOW faecal elastase demonstrates pancreatic insufficiency

Answer 427

Sweat test Excessive Na and Cl in the sweat Normal range = 1—30 mmolL CF = 60-125 mmol/L

Answer 428

Child and parents Paediatrician GP and primary care team Respiratory paediatrician Physiotherapist Specialist nurses Genetic counsellor for the family (i.e. brothers and sisters) Dietician Teachers

Answer 429

Chest physio 2x daily Physical exercise encouraged Continuous prophylactic oral antibiotics – usually flucloxacillin Rescue antibiotics when needed Nebulised DNase – decreases sputum viscosity to increase clearance Oxygen and CPAP may be needed long term Lung transplant

Answer 430

HIGH CALORIE INTAKE DIET ESSENTIAL – 150% of normal Oral enteric coated pancreatic replacement therapy with all meals and snacks Overnight feeding over gastrostomy Fat-soluble vitamin supplements (A, D, E, K)

Answer 431

The right side has the higher pressure Blood shunts through from the RA to the LA through the foramen ovale Most blood by this mechanism bypasses the lungs

Answer 432

First breath causes pulmonary blood flow to increase This causes a decrease in RA pressure and increase in Lap Causing the flap to close

Answer 433

5 Ts: Tetralogy of Fallot Transposition of the great vessels Tricuspid Atresia Truncus Arteriosus Total Anomalous Pulmonary venous connection (don’t really know what this is but I’ve put it in for completion) Complete AVSD is also cyanotic

Answer 434

Hyperoxic test (give them oxygen) If they get better 🡪 respiratory If they don’t 🡪 cardiac

Answer 435

Tetralogy of Fallot

Answer 436

Large VSD Overriding aorta Pulmonary stenosis Right ventricular hypertrophy

Answer 437

Boot shaped heart

Answer 438

Pulmonary hypertension Leading to an increase in RV pressure The RV blood can then flow through the VSD – causing a R🡪L shunt

Answer 439

Antenatally Or ID of murmur in first 2 months of life Some may present with severe cyanosis in the first few days of life

Answer 440

Severe cyanosis Hyper-cyanotic spells (rapid increase in cyanosis, inconsolable crying, breathlessness and pallor) Squatting on exercise

Answer 441

Ejection systolic murmur At the left sternal edge (pulmonary region)

Answer 442

Clubbing CONGENITAL HEART DISEASE IS A MAJOR CAUSE OF CLUBBING

Answer 443

Prostaglandins infusion to keep duct open until surgery Corrective surgery at 6-9 months of age

Answer 444

Aorta is connected to the RV Pulmonary artery is connected to the LV – meaning that deoxygenated blood gets pumped around the body = blue baby This is a DUCT DEPENDANT LESION

Answer 445

May be diagnosed antenatally Cyanosis may be present at birth Or present on day 1-2 of life when the duct closes Will be less severe if there’s an ASD or VSD allowing mixing NO MURMUR

Answer 446

Looks like an “egg on its side” Echocardiogram confirms diagnosis by demonstrating abnormal arterial connections

Answer 447

Prostaglandin infusion to keep the ductus arteriosus open

Answer 448

Balloon atrial septostomy – creates a hole between the 2 atria to allow mixing Arterial switch procedure – the aorta and PA are transected above the valves and switched over. Done ELECTIVELY later in life, not as an emergency straight after birth

Answer 449

Down’s syndrome All Down’s babies need an echo at birth

Answer 450

Either diagnosed antenatally Routine echo in a Down’s baby Cyanosis at birth Heart failure 2-3 weeks of life

Answer 451

Medical treatment for heart failure Surgical repair at 3-6 months

Answer 452

Neonatal cyanosis Treatment – shunting between the subclavian and pulmonary artery

Answer 453

Left to right shunts (VSD, ASD, PDA) These babies will either be breathless Or asymptomatic and be detected by heart murmur on examination

Answer 454

The 4 S’s: 1. Soft blowing murmur 2. ASymptomatic 3. Left Sternal edge 4. Systolic murmur only

Answer 455

SMALL VSD Large VSD is a much quieter murmur

Answer 456

Breathlessness Heart failure symptoms Failure to thrive

Answer 457

AS – ejection systolic murmur at upper RIGHT sternal edge PS – ejection systolic at LEFT sternal age

Answer 458

Circulatory collapse at 2 days old Absent femoral pulses (association with Turner’s syndrome)

Answer 459

Continuous murmur beneath the left clavicle

Answer 460

Premature babies

Answer 461

Dilated cardiomyopathy

Answer 462

Inherited Secondary to metabolic disease From a direct viral infection of the myocardium (myocarditis)

Answer 463

Symptomatic treatment – diuretics, ACEi, beta blockers (treat the symptoms of heart failure) Myocarditis usually resolves spontaneously Some children may need a heart transplant

Answer 464

Left to R shunts left untreated (volume overload) Coarctation AS, TR, MR Dilated cardiomyopathy Anything that affects all 3 layers – peri, myo and endocarditis, effusions

Answer 465

SOB and exercise intolerance Palpiations Chest pain Feeding problems (due to breathlessness) Poor/excessive weight gain As a rule in paeds – increase in HR + RR + creps = left heart failure Most common cause = large VSD

Answer 466

Peripheral oedema Liver enlargement (more sensitive than JVP) Causes – tricuspid regurg, pulmonary stenosis, large VSD etc. (anything that may cause fluid overload backwards through the IVC

Answer 467

Upper tract infection – involves the kidneys (pyelonephritis) and is associated with fever and systemic involvement Lower tract infection – involves the bladder (cystitis) and is less likely to be associated with a fever or the fever will only be low grade

Answer 468

May indicate an underlying structural pathology of the kidneys Recurrent kidney infections can cause renal scarring – predisposing to hypertension and chronic renal failure later in life

Answer 469

Escherischia Coli Usually via contamination from the bowel

Answer 470

Proteus – more common in boys, predisposes to formation of phosphate stones in the urine Pseudomonas – may indicate the presence of structural abnormality in the tract

Answer 471

Incomplete bladder emptying Vesico-ureteric reflux Structural abnormality e.g. horseshoe kidney, ureteric strictures Inadequate toilet hygiene

Answer 472

Post infectious scarring leading to: Hypertension Impaired GFR/chronic renal failure Pregnancy complications

Answer 473

Fever lethargy Irritability Vomiting and diarrhoea Poor feeding/failure to thrive Prolonged neonatal jaundice Septicaemia Febrile convulsions (> 6 months)

Answer 474

Dysuria (painful urination) Loin pain or abdominal pain Increased frequency of urination Fever (with or without rigors) Lethargy Anorexia Febrile convulsions Recurrent enuresis (wetting themselves) Haematuria

Answer 475

URINE SAMPLE: Babies – nappy pads, clean catch, in and out catheter, suprapubic aspiration from the bladder using ultrasound guidance Children – midstream urine sample. Should be tested straight away and if not then they must be refrigerated Ultrasound scan Functional scanning – DMSA to look for scarring Micturating cystourethrogram (MCUG) – looks for reflux

Answer 476

Antibiotics – usually 3 day course of trimethoprim or 5 days of co-amoxiclav Fluids – keep hydrated to support the kidneys Analgesia (calpol etc.) Adjust antibiotics based on sensitivites once urine sample is returned

Answer 477

Education about perineal hygiene (wipe front to back) Regular voiding High fluid intake to produce high urine output Ensure complete bladder emptying (try a second time after a minute or 2) Prevent and treat constipation Antibiotic prophylaxis in those under 2 with severe reflux (trimethoprim)

Answer 478

Surgery is an option for reflux Long term low dose antibiotic prophylaxis Circumcision Annual BP checks Regular assessment of kidney function and growth

Answer 479

Transient proteinuria – fever, exercise Orthostatic (postural) Nephrotic syndrome Hypertension Tubular proteinuria Increased glomerular perfusion pressure

Answer 480

Heavy proteinuria Hypoalbuminaemia Oedema Hyperlipidaemia

Answer 481

Congenital Steroid sensitive Steroid resistant

Answer 482

Congenital – Finnish people Steroid sensitive – boys, Asians, atopic children

Answer 483

Minimal change disease/minimal change glomerulonephritis

Answer 484

HSP and other vasculitides SLE Infections (e.g. malaria) Allergens (e.g. bee sting)

Answer 485

Frothy urine – caused by significant proteinuria Oedema – pitting and gravitational Periorbital oedema (particular on waking) 🡪 earliest sign Scrotal, vulval, leg and ankle oedema Ascites Breathlessness due to pleural effusions and abdominal distention

Answer 486

Present in first 3 months of life Rare, very serious ESRF and need for dialysis (if survival past first 6-12 months) High mortality

Answer 487

Age 1-10 years Often precipitated by respiratory infections No macroscopic haematuria Normal BP Normal complement levels Normal renal function No features to suggest nephritis Responds to steroids Normally shows “minimal change” on histology

Answer 488

Recurrent relapses throughout childhood 5% of cases continue into adult life Treat with steroids If continues through whole life - may need steroid sparing agent such as methotrexate

Answer 489

Older children Haematuria Low complement levels (the complement levels build up in the kidneys, may precede SLE) Associtaed with hepatitis B

Answer 490

30% respond to ESRF in 5 years 20% respond to cytotoxic medications Most spontaneously remit in 5 years

Answer 491

Urine dipstick – looking for protein in particular FBC and ESR U&E, creatinine, albumin Complement levels – C3 and C4 Antistreptolysin O titre and throat swab Urine MC&S Urine sodium concentration Hepatitis B antigen

Answer 492

Standard course of oral prednisolone for first episode Treat with steroids again for subsequent episodes Consider MTX if recurs all the time Restrict sodium and water intake and use diuretics until oedema settles Pen V Measles and chickenpox immunity

Answer 493

Manage oedema – diuretics, salt and fluid restriction, ACEis Could try a cytotoxic medication such as methotrexate

Answer 494

Glomerular haematuria – brown urine, deformed red cells, presence of casts, often accompanied by proteinuria Lower urinary tract – red urine, occurs at the beginning or end of urinary stream, not accompanied by proteinuria

Answer 495

Urinary tract infection NB haematuria will be alongside all the other symptoms associated with UTI (loin pain, dysuria, increased frequency)

Answer 496

Infection Trauma Stones (esp. if there’s a family history) Sickle cell disease Bleeding disorders Renal vein thrombosis Malignancy – Wilm’s tumour

Answer 497

Acute glomerulonephritis Chronic glomerulonephritis IgA nephropathy (Berger’s disease) Familial nephritis Post strep glomerulonephritis Vasculitis – HSP (triad of rash, abdominal pain, arthritis) Goodpasture’s syndrome – anti-GBM disease – very rare, will also cause haemoptysis

Answer 498

Urine dipstick Urine MC&S Urine protein and calcium USS – kidneys and urinary tract FBC, U&E, platelets, clotting screen Creatinine and albumin Throat swab and anti-streptolysin O titre ESR & complement levels

Answer 499

Recurrent macroscopic haematuria Suspicion of familial nephritis Abnormal renal function Abnormal complement levels Proteinuria

Answer 500

Aka acute nephritis, glomerular nephritis Haematuria (often macroscopic) Proteinuria – varying degree Impaired GFR – rising creatinine Salt and water retention – hypertension, oedema

Answer 501

Usually follows a strep throat or skin infection (post streptococcal glomerulonephritis) Vasculitis – HSP, SLE, Wegner’s, PNA IgA nephropathy (Berger’s disease) Goodpasture’s syndrome Familial nephritis (Alport’s syndrome, X-linked)

Answer 502

Group A beta haemolytic strep (strep pyogenes)

Answer 503

FBC U&E – increased urea and creatinine, hyperkalaemic acidosis C3 and C4 – may be low Anti-streptolysin O titre – raised Throat/skin swabs Urinalysis – haematuria, raised protein on dipstick, RBCs

Answer 504

Fluid and electrolyte balance Treat hypertension – diuretics Correction of other imbalances – potassium, acidosis Penicillin – treatment of streptococcal infection if needed The nephritis usually setles by itself, may need steroids Monitor urine output and creatinine

Answer 505

Undescended testes The testes has been arrested along its normal pathway of descent

Answer 506

Prematurity

Answer 507

Retractile Palpable Impalpable

Answer 508

Can be manipulated into the bottom of the scrotum But as soon as you let go it retracts into the inguinal region – pulled up by the cremaster muscle Usually present later as hard to notice on neonatal check Eventually the testes should permanently reside in the scrotum But does require follow-up to check

Answer 509

Can be palpated in the groin But cannot be manipulated into the scrotum Occasionally testes can be ectopic (outside the normal line of descent) so may be felt in the perineum or femoral triangle

Answer 510

No testis can be felt on detailed examination The testis may be intra-abdominal, in the inguinal canal or completely absent

Answer 511

Genital examination – in a warm room, warm hands, relaxed child. Try and “milk” the testes into the scrotum Ultrasound scan Hormonal investigations Karyotype Laparoscopy – investigation of choice to look for an impalpable testicle (see whether it’s completely absent or just abdominal)

Answer 512

Orchidopexy – surgical placement of the testis in the scrotum If testicles absent altogther – start on testosterone replacement therapy around the age of 10 (start of puberty) and consider testicular prosthesis for cosmetic purposes

Answer 513

Fertility – is at risk if the testes are not within the scrotum Malignancy – greater risk in undescended testes Cosmetic and psychological reasons

Answer 514

Tender, red, painful testicle Abdominal pain Vomiting

Answer 515

Neonatal and puberty are the peak ages for incidence But can happen at any age So have a low threshold for investigation

Answer 516

Doppler ultrasound scan – demonstrates decreased blood flow (as torsion of the spermatic cord results in testicular ischaemia) Surgical intervention – emergency – needs operating to un-tort within 6 hours If the testicle is dead – orchidectomy

Answer 517

In any child whose condition does not resolve or respond to treatment in the normal way Do not be afraid to challenge the original diagnosis If in doubt – retake a full history

Answer 518

Very common – affects up to 50% of children Most commonly due to a self-limiting benign cause (i.e. cough/cold)

Answer 519

HIV Autoimmune conditions Glycogen storage disorder Malignancy

Answer 520

Enlarging node without clear infective cause Persistent Unusual site e.g. supraclavicular – typically indicated mediastinal mass If there’s associated signs and symptoms that are worrying (i.e. anaemia, bruising) Fever, weight loss, enlarged liver/spleen If abnormal CXR or respiratory symptoms

Answer 521

Acute lymphoblastic leukaemia (ALL) Followed by brain tumours

Answer 522

Precursors to B and T cells So affects the blood and bone marrow

Answer 523

About 80% cure rate Good prognostic indicators: Age 2-10 Female WCC < 50 No CNS disease

Answer 524

Down’s syndrome Immune compromisation – HIV, organ transplant and therefore on immunosuppressants

Answer 525

Bone marrow infiltration Organ infiltration With leukaemic blast cells

Answer 526

SYMPTOMS OF PANCYTOPENIA: Infections (due to neutropenia) Lethargy and pallor (due to anaemia) Abnormal bruising, petechiae, nose bleeds (due to pancytopenia) ALWAYS ask about the other symptoms if they present with any of these Malaise Anorexia Hepatosplenomegaly Lymphadenopathy (lumps and bumps) Bone pain (due to bone marrow infiltration)

Answer 527

CNS infiltration – headaches, vomiting, nerve palsies Testicular enlargement

Answer 528

Insidious onset over around 4 weeks May have had a non-specific prodrome for several months

Answer 529

Bone marrow aspirate

Answer 530

BLOOD COUNT showing: Low Hb (anaemia) Thrombocytopenia Evidence of circulating blast cells WCC may be up or down Neutropenia LP CXR

Answer 531

Induction Consolidation and CNS treatment Intensification Maintenance (2 years for girls, 3 years for boys – as boys have a higher incidence of recurrence, particularly in the testes)

Answer 532

Vincristine Plus a steroid such as dexamethasone

Answer 533

Haemopoetic stem cell transplant This can be given in high risk patients in the first episode of disease Or for those who relapse

Answer 534

Psychological impact of childhood cancer – always mental health screen them Family and social impact Fertility – offer freeze eggs/sperm before treatment Growth impact CNS development Cardiac and renal toxicity Delayed puberty Risk of recurrence is a large worry

Answer 535

Reed-Sternberg cells

Answer 536

T-cell malignancies – mediastinal mass plus bone marrow infiltration (anaemia, bruising, infections) B-cell malignancies – localised lymphadenopathy in head, neck or abdomen Adominal disease – pain, mass, intussusception/obstruction

Answer 537

Painless lymphadenopathy – most commonly in the neck LARGER AND FIRMER lymph nodes than in benign lymphadenopathy Often has a long history Systemic ‘B’ symptoms: Sweating Pruritus Weight loss Fever

Answer 538

NHL – stage with CT/MRI, bone marrow aspirate, CSF sample Hodgkin’s – diagnostic lymph node biopsy, intra-adominal disease assessed radiologically

Answer 539

NHL - multi-agent chemotherapy Hodgkin’s – chemo +/- radiotherapy About 80% cure rate

Answer 540

In adults - brain tumours are normally secondary from another cancer elsewhere In children – brain tumours are almost always primary

Answer 541

Astrocytoma – 40% (this includes glioblastoma multiforme) Medulloblastoma - 20% Ependymoma – 8% Brain stem glioma – 6% Craniopharyngioma – 4%

Answer 542

Broadly – signs of Raised ICP and focal neurological signs of wherever the tumour is located

Answer 543

Headache (early morning, worse when lying down) – raised ICP Vomiting (especially when waking in the mornings) Papilloedema Squint secondary to 6th nerve palsy Nystagmus Ataxia Seizures/epilepsy Personality/behavioural change Headaches + personality change 🡪 consider raised ICP

Answer 544

If there’s eye signs – papilloedema, decreased acuity, visual loss If other neurological signs develop (nystagmus etc.) If recurrent and early morning If associated with vomiting If there’s short stature/decelerated growth If there’s symptoms of diabetes insipidus or other endocrine disorders If < 3 years If the child has NF1

Answer 545

MRI scans are the best way to look at tumours Do not perform LP if signs of raised ICP

Answer 546

Surgery is the best option if the tumour is operable Radiotherapy – stereotactic gamma knife is worth a try Chemotherpay

Answer 547

Outcome depends on where the tumour was and how well the surgery cleared it/how much healthy brain tissue was removed alongside the tumour Survivors may face – neurological disability, growth problems, endocrine problems, and neuropsychological problems

Answer 548

Tumours of neural crest tissue In the ADRENAL MEDULLA AND SYMPATHETIC NERVOUS SYSTEM NOT BRAIN TUMOURS Can be benign (ganglioneuroma) or malignant (neuroblastoma)

Answer 549

Most common < 5 years

Answer 550

Abdominal mass – if the tumour is in the adrenals (most common site) Mass anywhere along sympathetic chain 🡪 may lead to spinal cord compression Miserable child with big abdomen 🡪 think neuroblastoma

Answer 551

MRI/CT Urinary catecholamines - raised Biopsy Bone marrow sampling MIBG – metaiodobenzynl guanidine

Answer 552

Surgery – for localised disease Chemo – advanced disease (symptoms of advanced disease = bone pain, pancytopenia, weight loss, malaise)

Answer 553

Wilm’s tumour 80% present < 5 years Rare > 10 years Originates from embryonal renal tissue

Answer 554

FAMILY HISTORY Wilm’s tumour susceptibility gene

Answer 555

Large abdominal mass Otherwise well child PAINLESS haematuria Sometimes hypertension, poor appetite, poor weight gain 5% have bilateral disease at diagnosis

Answer 556

USS/CT/MRI Staging to assess for mets – usually in the lungs

Answer 557

Chemotherapy for 6 months to shrink tumour(s) Nephrectomy – full if only one kidney, partial if both kidneys affected Radiotherapy 80% cure

Answer 558

Rhabdomyosarcoma Usually in the head/neck

Answer 559

Osteogenic sarcoma Ewing’s sarcoma Both are more common in males

Answer 560

Persistent localised bone pain Mass Limbs most common site Well child at diagnosis

Answer 561

X-Ray – mass, destruction, periosteal new bone formation CT scan to determine extent of diesease

Answer 562

Neoadjuvant chemotherapy Surgery – try and remove the tumour but preserve the limb, amputation avoided where possible Ewing’s – radiotherapy manages local disease

Answer 563

Malignant tumour of the retinal cells of the eye Can affect one or both eyes All bilateral tumours = hereditary 15% of unilateral cases = heriditary Autosomal dominant inheritance (incomplete penetrance) Retinoblastoma susceptibility gene on chromosome 13

Answer 564

Abnormal red reflex seen in flash photography WHITE PUPILLARY REFLEX replaces the normal red one Screened for in NIPE Can also present with decreased visual acuity and nystagmus

Answer 565

Full ocular examination – fundoscopy Biopsy Genetic testing Genetic counselling

Answer 566

Aim of treatment = get rid of cancer, preserve as much vision as possible May need to remove the eye (enucleation) in advanced cases Chemotherapy Local laser treatment Most are cured but visually impaired Large risk of secondary malignancy (especially sarcoma)

Answer 567

Abdominal distention/mass Sometimes jaundice Primary liver tumours in neonates = haemangioma

Answer 568

USS/CT/MRI Serum alfafetoprotein

Answer 569

Lump – sacrococcygeal region or gonads Investigate - serum AFP and serum BCG Treatment – chemo, good outcome

Answer 570

Abnormal prolieration of histiocytes (stationary phagocytic cells in connective tissue) Not truly malignant Can be aggressive and responds to chemotherapy

Answer 571

Spectrum of disease from localised bone lesions = systemic /vague presentation Bone lesions – pain, swelling, fractures (often in skull) Diabetes insipidus – due to skull disease Rash Soft tissue involvement (gums, ears, spleen, LNs, bone marrow) Recurrent discharging ear

Answer 572

XR – characteristic lytic lesions with well defined border (often in skull) Full skeletal survey to identify multiple lesions

Answer 573

Bone lesions – biopsy Diabetes insipidus – long-term treatment with desmopressin usually required Systemic - chemotherapy, variable prognosis

Answer 574

Iron deficiency

Answer 575

Has a higher affinity for oxygen than adult Hb so the foetus can survive the lower oxygen concentrations in the womb Switches gradually to HbA By 6 months, most of the HbF is HbA

Answer 576

Not fully developed = insufficient EPO production Protein content of breastmilk not sufficiency for haemopoesis in premature infants Signs and symptoms - apnoea, poor weight gain, pallor, decreased activity, tachycardia

Answer 577

Reduced/impaired red cell production (iron deficiency, red cell aplasia) Increased red cell destruction – haemolysis Blood loss – relatively uncommon in children

Answer 578

Young infants – slow feeding/may seem breathless Children – tire easily Pale conjunctiva, tongue or palmar creases Irritability Tachycardia Pica – eating non-food materials Iron deficiency – behavioural problems/reduced intellectual function Malabsorption picture Hepatomegaly, splenomegaly, jaundice – if haemolysis

Answer 579

Full blood count Haematinics Reticulocyte count (high circulating reticulocytes indicated haemolysis) Bone marrow investigation

Answer 580

Jaundice at < 24 hours is often due to haemolysis Do a Coomb’s test (ABO incompatibility/Rh incompatibility)

Answer 581

Dietary advice (red meat, brocolli, kale) Oral iron supplement

Answer 582

Antibiotic prophylaxis – twice daily penicillin against pneumococcus Treatment of acute crises – analgesia, hydration, oxygen Exchange transfusion – acute chest, stroke, priapism Treatment of chronic problems – hydroxyurea, hydroxycarbamide (these increase the level of feotal haemoglobin which replaces the sickle haemoglobin) Really dangerous if they get Parvovirus B19 (slapped cheek) – causes aplastic crisis. Need good hydration and oxygen

Answer 583

Thalassaemia = they are missing part of the haemoglobin molecule Treatment - regular blood transfusions, frequency depends on severity of the disease Iron chelation to prevent iron overload (which can lead to liver failure, cardiomyopathy, heart failure, pancreatic dysfunction) Bone marrow transplant as a last resort

Answer 584

Bone marrow failure – the bone marrow is not producing any blood cells Characterised by reduction or absence in all 3 main lineages in the bone marrow – leading to peripheral pancytopenia (Platelets come from megakaryocytes)

Answer 585

Idiopathic Viruses – hepatitis Drugs – chemotherapy, sulphonamides (antibiotics) Toxins - benzene, glue Fanconi anaemia – autosomal recessive, short stature, abnormal radii and thumbs, micropthalmia, skin lesions, renal malformations Schwachman-Diamond syndrome – autosomal recessive, bone marrow failure, pancreatic exocrine failure, skeletal abnormalities

Answer 586

Anaemia Infection Bruising and bleeding Symptoms of pancytopenia so DDX = blood cancers such as leukaemia and lymphoma

Answer 587

Full blood count Bone marrow studies

Answer 588

Address underlying cause Supportive treatment (fluids, antibiotics) – wait for them to return to full health i.e. if it’s due to chemo etc. Bone marrow transplant if severe

Answer 589

Vitamin K deficiency Liver disease – cannot produce clotting factors or store vit A, D, E and K effectively Thrombocytopenia – idiopathic thrombocytopenic purpura following viral infections in young children

Answer 590

Haemophilia A (factor 8 deficiency) and B (factor 9 deficiency) – X linked recessive, more common in boys, usually presents with recurrent spontaneous bleeds into joints and muscles around 1 year of age Von-Willebrand’s disease – autosomal dominant, presents with mucosal bleeding (e.g. epistaxis, menorrhagia) or excessive bleeding after surgery

Answer 591

Turner’s syndrome 45, XO They don’t have the protection from the extra X chromosome

Answer 592

Haemophilia – tends to cause big bleeds into joints VWD – mucosal bleeding (epistaxis, gum bleeds, menorrhagia)

Answer 593

Petechiae/purpura Easy bruising

Answer 594

Purpura Arthritis Abdominal pain

Answer 595

FBC and blood film Prothrombin time – measures factors 2, 5, 7, 10 Activated partial prothrombin time Thrombin time Quantitative fibrinogen assay D-Dimers Biochemical screen including renal and liver function

Answer 596

10 9 7 2 (1972)

Answer 597

Vitamin K injection – given to all neonates, supplements given in children Diagnose and treat any liver disease Platelet infusion if thrombocytopenia

Answer 598

Recombinant factor 8 for A Recombinant factor 9 for B

Answer 599

Mild – desmopressin Severe – plasma derived factor 8 concentrate

Answer 600

“Thrombophilias”: Protein C deficiency Protein S deficiency Anti-thrombin deficiency Factor V Leiden deficiency

Answer 601

Catheter related – i.e. during an investigation DIC Hypernatraemia Polycythaemia (due to congenial heart disease or placental insufficiency in utero) Malignancy SLE

Answer 602

Inherited conditions – present with venous thrombosis in 2nd or 3rd decade Thrombosis of cerebral vessels = stroke ”purpura fulminans” – widespread haemorrhage and purpura into the skin in the neonatal period

Answer 603

Any child with venous thrombosis Ischaemic skin lesions Neonatal purpura fulminans Positive family history of purpura fulminans Known family history of clotting disorder

Answer 604

Warfarin NOACs

Answer 605

Inattention Impulsivity Hyperactivity NB – these symptoms occur in every child occasionally, but when they are persistent and impact on daily function, more investigations are needed

Answer 606

Boys 4x more likely than girls Prematurity Low socio-economic status Other psychiatric disorders – Asperger’s, Tic, ODD CNS insults – perinatal infections, meningitis, fetal alcohol syndrome

Answer 607

Easily distracted Does not appear to listen when spoken to Forgetful in daily activities Loses important items Finds it difficult to follow instructions/complete tasks Makes careless mistakes

Answer 608

”can’t sit still” Runs or climbs excessively Appears to be constantly “on the go” and “driven by a motor” Talks excessively Cannot perform leisurely activities quietly

Answer 609

Blurts answers before questions are completed Has difficult awaiting turn Interrupts or intrudes on others Teenagers get into big trouble for impulsive behaviour 🡪 more likely to have car accidents/get pregnant

Answer 610

Clinical diagnosis - take a history Assessment in school and at home (need to exhibit the behaviour in more than 1 domain) Information from teachers, school reports, family members, previous doctors reports and notes

Answer 611

Education ADHD parenting program Support from school Medications – methylphenidate (a CNS stimulant)

Answer 612

Impaired social interaction Speech and language disorder Imposition of routines, with ritualistic and repetitive behaviour

Answer 613

Lack of desire to communicate at all Repeats speech – echolalia Won’t hold eye contact Poor body language Unable to understand sarcasm Doesn’t play imaginatively Has obsessions and rituals Regression in development – i.e. they started to talk and then stopped Does not like change

Answer 614

MDT approach – liaise with school, nurses, GP, educational classes for parents Using things like picture-based timetables they can tick off when they do something to get them through the day Visual clues avoid anxiety Manage associated co-morbidities

Answer 615

Body weight < 15% expected for age and height Self-induced weight loss – avoiding food, vomiting, laxatives, excessive exercise Body image distortion HPG axis disorder – delayed puberty, loss of sexual interest and potency

Answer 616

Female gender High achiever Friendship issues Stressful life events to trigger the process Middle class Complex family dynamic Bereavement Domestic/ sexual abuse Being overweight as a child

Answer 617

Cognitive effects Muscle wasting – eventually affects the heart leading to CCF, can’t squat down and stand up without touching anything Osteoporosis Extra hair growing Constipation Can’t maintain body temperature Teeth – dental erosions due to vomiting

Answer 618

SCOFF questionnaire: 1. S – do you ever make yourself sick? 2. C – do you feel like you lose control when you eat? 3. O – have you lost more than One stone in a 3 month period? 4. F – do you believe yourself to be fat? 5. F – does food dominate your life?

Answer 619

MARSIPAN tool Externalising the anorexia is a first line step of treatment – name the anorexia, dissociate it from the young person and try and address it as a separate entity Aim for slow and steady weight gain in the community Family approach and individual therapy MDT approach – GP, CAHMS counselling, dietician Medications – SSRIs, antipsychotics (if they have body dismorphic disorder) and vitamin replacements as required

Answer 620

MARSIPAN tool Externalising the anorexia is a first line step of treatment – name the anorexia, dissociate it from the young person and try and address it as a separate entity Aim for slow and steady weight gain in the community Family approach and individual therapy MDT approach – GP, CAHMS counselling, dietician Medications – SSRIs, antipsychotics (if they have body dismorphic disorder) and vitamin replacements as required

Answer 621

Risk of RE-FEEDING SYNDROME When the body is starved for a long time giving glucose can be dangerous The body starts taking up glucose and excreting potassium and sodium Causes re-feeding hepatitis

Answer 622

1/3 recover 1/3 relapse and remit 1/3 chronic lifelong illness

Answer 623

Moles = naevi Turner’s syndrome

Answer 624

Stephen Johnson syndrome Disorder usually caused by infections or drugs Causes peeling of the skin, hands and mucosal changes High mortality

Answer 625

HSP rash looks very much like meningococcal rash (non-blanching, purpuric) But the child will be well and afebrile compared to a septic child Meningococcal rash will be all over the body HSP rash generally sticks to the lower limbs and buttocks HSP triad – abdo pain, purpura, arthritis If in doubt – treat as meningococcal sepsis

Answer 626

Acanthosis nigricans This child will also be obese

Answer 627

In babies – tends to be on their face and trunk Past age of 1 – can be generalised but tends to occur in the creases

Answer 628

It causes severe neurological dysfunction which leads to severe learning difficulties later In life Relatively common – affects 1 in 4000 births

Answer 629

Worldwide – iodine deficiency UK – mal-descent ent of thyroid/absent thyroid Consanguinous families – dyshormonogenesis (inborn error of thyroid hormone synthesis)

Answer 630

PICKED UP ON GUTHRIE TEST – antenatal screening But if not picked up Failure to thrive Jaundice Feeding problems Hoarse cry Goitre Delayed development Etc.

Answer 631

Lifelong oral replacement of thyroxine (if due to maldescent, athyrosis or dyshormonogenesis) Titrate dose to maintain normal growth `

Answer 632

Normal TSH levels

Answer 633

Autoimmune thyroiditis (Hashimoto’s?) Symptoms the same as in adult hypothyroidism

Answer 634

Grave’s diesease More common in girls More common in people with another autoimmune disease (Type 1 diabetes, coeliac disease) Symptoms same as in adult thyroid disease

Answer 635

Carbimazole Propylthiouracil Either by a dose titration or block and replace with thyroxine

Answer 636

Acts to lower the blood glucose level Does this by stimulating glucose uptake from the blood into – muscle, kidney and fat cells Targets the liver to convert glucose into glycogen

Answer 637

Either picked up by chance on a random plasma glucose Or – polydipsia, polyuria, weight loss, fatigue

Answer 638

Smell of acetone on breath (pear drops) Vomiting Dehydration Abdominal pain Hyperventilation due to acidosis (Kussmaul breathing) Hypovolemic shock Drowsiness Coma

Answer 639

Hypokalaemic Hypochloraemic Metabolic acidosis

Answer 640

Aggressive fluid resuscitation before anything else Insulin Potassium Glucose Hourly obs watch closely and rehydrate slowly

Answer 641

Cerebral oedema

Answer 642

Irritable Nauseous Shaky Sweaty Pale Dizzy Confused Drowsy Odd behaviour Hypo seizure – convulsions can occur

Answer 643

Hypo unawareness Rebound hypoglycaemia Rebound hyperglycaemia Dawn phenomena – morning rise in blood sugar

Answer 644

Random plasma glucose ≥ 11.1 mmol/l Fasting plasma glucose ≥ 7.0 mmol/l HbA1c > 6.5% (or > 48 mmol/l)

Answer 645

Check BM to confirm Glucose tablets/glucogel / 3 jelly babies Follow up with longer acting carbohydrate e.g. sandwhich Check BMs again after 15 mins

Answer 646

Do not attempt to give anything by mouth Glucagon – SC or IM injection (0.5mg if < 5 years, 1mg if > 5 years) Wait 10 mins When conscious give longer acting carb

Answer 647

Hip Then leg Then knee Then thigh Then foot (least likely to be limping due to a foot problem)

Answer 648

Transient synovitis (post-infectious) Slipped upper femoral epiphysis Reactive arthritis DDH Septic arthritis Duchenne’s Osteomyelitis Perthe’s disease – avascular necrosis of the femoral head Cancer – Ewing’s, osteogenic sarcoma

Answer 649

Limping unhappy child Post viral- after cough, cold Limp < ROM NB – HIP PROBLEMS CAN OFTEN PRESENT AS KNEE PAIN

Answer 650

FULL HISTORY – including recent illnesses such as coughs and colds EXAMINE FROM HEAD TO TOE Urgent inflammatory markers Ultrasound the joint to look for thickening of capsule or effusion

Answer 651

Traumatic acute limping child History important Age 7-16 years Not weightbaring Externally rotated hip KNEE PAIN Consider congenital insensitivity to pain – these patients can dislocate joints without realising

Answer 652

Septic arthritis and oesteomyelitis They will be systemically unwell and pyrexic << ROM (limb tends to be completely immobile)

Answer 653

History Examination – red, hot, swollen extremely tender joint FBC, WCC, ESR, CRP X ray the affected joint USS joint capsule Blood cultures Joint aspiration for cultures

Answer 654

Infants – group B strep, staph aureus, coliforms Children up to 4 – staph aureus, pneumococcus, haemophilus influenza Children >4/adolescents – staph aureus, gonococcus s

Answer 655

Perthe’s disease SUFE DDH (developmental dyplasia of the hip) Spinal problems Juvenile idiopathic arthritis Bone tumours – there is often a PALPABLE MASS Foot deformities

Answer 656

4-8 years old Unilateral hip pain Limp Family history < ROM, < internal and external rotation There is often apparent shortening of one of the legs

Answer 657

9-16 years old Chronic (> 3 weeks) Pain – knee, thigh, hip STEROTYPE – MALE, LOWER SOCIOECONOMIC STATUS, PARENTS SMOKE Slightly overweight child Comes on gradually More common in children with endocrine issues Surgical emergency

Answer 658

Should be picked up on NIPE but can be a late presenter Leg length discrepency Reduced abduction Otherwise well Typically “waddle” when they walk

Answer 659

Galleaze sign Child is laying down on the bed with their feet flat against the bed Positive test = knees are different heights Limited abduction on dislocated hip

Answer 660

Diagnosis of exclusion Growing pains are common Females > males Usually bilaterla DO NOT CAUSE LIMP and won’t have any systemic features Last thing you reach for if you cannot explain the pain

Answer 661

Pain? Unwell in general? Onset – sudden or gradual? What type of limp? Can the problem be localised? Has it improved, got worse or stayed the same?

Answer 662

1 = heel off 2 = flat 3 = toe off

Answer 663

Pathology = abductor dysfunction Can be caused by - hip dysplasia, fracture that hasn’t healed, neurologcal injury, low grade long-term bone infection

Answer 664

Gower test

Answer 665

Aspiration/drainage of the pus in the joint Antibiotics – often flucloxacillin because the infection is often caused by staph aureus

Answer 666

Onset before 16th birthday No identified underlying cause Persistent joint swelling/inflammation Lasting at least 6 weeks (i.e. it’s not reactive arthritis)

Answer 667

Due to the inflammatory nature of JIA, this will be the same as the joint features seen in RA Loss of joint space Joint deformity Soft tissue swelling Periarticular osteopenia

Answer 668

Persistent joint swelling Limping/functional disability Pain Joint stiffness (particularly in the morning) Decreased ROM Joint deformity Warmth Colour change

Answer 669

Oligo articular – affects < 4 joints, big joints mainly Polyarticular – affects > 4 joints, small joints mainly Psoriatic – associated with nail pitting, dactylitis, family history Entethesis related Systemic arthritis – generalised inflammatory signs all over the body eg. Fever, rash, lymphadenopathy, hepatosplenomegaly

Answer 670

GAIT – screening test for all sorts of joint problems

Answer 671

Oligo - inject the affected joints with steroid Poly – system therapy - methotrexate is the safest and best option, NOT steroids in kids After 3 months if no response – start biologic (e.g. infliximab, adalamumab

Answer 672

50% “grow out of it” and will no longer need treatment 50% will have chronic lifelong disease

Answer 673

1 or more vertebral crush fractures 2 or more long bone fractures by the age of 10 3 or more by 19 Bone density less than 2.5 SDs below the mean

Answer 674

Inherited/congenital: Osteogenesis imperfecta Haematological problems Acquired: Drug induced – particularly steroids (even ICS for asthma) Endocrinopathies – hypoparathyroidism (low calcium) Malabsorption Immobilisation e.g. disabilities Inflammation and inflammatory disorders

Answer 675

Type 2 is fatal Child dies very early because the chest is too small to allow breathing They have a lot of rib fractures (sometimes caused by the birth process) and their lungs don’t function at all

Answer 676

Autosomal dominant Defects in type 1 collagen genes – the type of collagen in bones

Answer 677

Bone fragility, fractures and deformity Bone pain Impaired mobility Poor growth – much smaller than children of the same age Deafness Poor muscle mass Hernia Valvular prolapse, aortic dissection Lovely happy smiley children compared to what you’d expect

Answer 678

Blue tinted sclera

Answer 679

Wormian bones (wiggly black lines in the skull) – feels like bubble wrap under your fingers if you feel their skull Bent bones - e.g. bent tibia Bowing of the femur Complete chest collapse in type 2 OI – fatal

Answer 680

Child and their family Metabolic bone doctors Pain team Physiotherapy Occupational therapy Specialist nurse Dietician School and teachers

Answer 681

Bisphosphonates – pamidronate Prefer to give IV These help increase lumbar spine and total body bone mass

Answer 682

”Bent bones” e.g. bow legs due to under mineralisation of the bones Occurs due to vitamin D deficiency

Answer 683

Insufficient maternal vitamin D during pregnancy Lack of child’s exposure to sunlight Dark skin Lack of vitamin D in diet – prolonged un-supplemented breastfeeding

Answer 684

Increases calcium absorption from the gut Increases calcium release from the bone Decreases calcium excretion by the kidneys Role in immune function/tolerance

Answer 685

Bowed leg deformity Hypocalcaemia convulsions Gross motor delay Incidental X-Ray finding Swollen ankle Carpopedal spasm

Answer 686

History Examination Biochemistry – serum calcium levels low, raised PTH levels XR – bowed legs, splayed metaphyses, frayed metaphyses

Answer 687

Vitamin D supplement NOT the active metabolite – give them the inactive form because as long as they don’t have kidney or liver disease they will be able to convert it themselves

Answer 688

A neurological event causing a sudden disturbance of neurological function due to excessive discharge of neurones

Answer 689

Epilepsy Febrile convulsions Metabolic – hypo seizures, hypocalcaemia Head trauma Infection - meningitis, encephalitis, brain abscess Poisons/toxins – amphetamines/stimulants Iatrogenic – post brain surgery

Answer 690

6 months – 6 years They usually have a genetic predisposition

Answer 691

Breath holding attacks – cries, holds breath, goes blue, recovers quickly Reflex anoxic seizures – triggered by pain, cold food, fright, fever Syncope Migraine Benign paroxysmal vertigo - associated with nsytagmus, unsteadiness, viral labrynthitis Arrythmias Non-epileptic attack disorders Fabricated by a parent

Answer 692

Generalised – discharge arises from both hemispheres (absence, myoclonic, tonic, tonic-clonic, atonic) Focal – seizures arise from one part of the brain (so will only affect for example their right hand)

Answer 693

Strange warning feelings/aura - smell, taste, visual, ‘rising sensation’ Automatisms – lip smacking, plucking clothing, pacing De ja vu and jamais vu Impaired consciousness – longer duration than a typical absence seizure

Answer 694

WITNESS HISTORY is important – it is often a clinical diagnosis History from patient esp. family history and birth/development EEG Blood tests Metabolic investigations

Answer 695

Explanation of diagnosis MDT approach – family, school, doctors, specialist nurses, OT Anti-epileptic medications – valproate, lamotrigine, carbamazepine

Answer 696

Carbamazepine

Answer 697

A disorder of movement and posture due to a non-progressive lesion of motor pathways in the developing brain The symptoms develop over time as the child starts to develop – the specific deficits due to the non-progressive lesion becomes apparent

Answer 698

Epilepsy Squints Visual impairment Speech and language disorders due to hearing problems Behaviour problems Feeding problems Joint contractures Hip subluxation Scoliosis

Answer 699

Antenatal (80%) – genetic syndromes, congenital malformations, congenital infections Hypoxic-ischaemic injury at birth (10%) Postnatal (10%) – intraventricular haemorrhage in premies, meningitis, encephalitis, trauma, NAI, hydrocephalus, kernicterus du to hyperbilirubinaemia

Answer 700

Abnormal limb tone (head and trunk hypo, limbs hyper) Abnormal limb and trunk posture Delayed motor milestones Feeding difficulties – oromotor incoordination, slow feeding, gagging, vomiting Abnormal gait Hand preference before 12 months of age Persistent primitive reflexes beyond 6 months of age

Answer 701

Spastic (70%) Ataxic hypotonic (10%) Dyskinetic (10%) 10% will have a mixed picture of this

Answer 702

UMN pathways affected – pyramidal or corticospinal Features – increased limb tone (spasticity), brisk deep tendon reflexes, extensor plantar response (positive Babinski sign), increased tone may suddenly yeild under pressure (clasp knife)

Answer 703

Hemiplegic Quadriplegic Diplegic

Answer 704

They will have one “bad side” Unilateral involvement of arm and leg Arm usually worse Face spared Tip-toe walking Fisting and flexion of the affected arm

Answer 705

All 4 limbs affected Arms usually worse Trunk involved – extensor posturing, poor head control, low central tone Seizures Microcephaly Learning difficulites

Answer 706

All 4 limbs affected But LEGS WORSE – so arm function appears relatively normal Walking is abnormal

Answer 707

Symmetrical Early trunk and limb hypotonia Poor balance Delayed motor development Signs – ataxic gait, intention tremor Cerebellar signs – so shows cerebellum is affected

Answer 708

Fluctuating tone Involuntary movements Chorea, dystonia Intellect unimparied Basal ganglia are affected (extra-pyramidal)

Answer 709

MRI brain – helps identify cause of CP CLINICAL DIAGNOSIS – assessment of pattern of tone in the limbs and trunk, posture, hand function, gait, reflexes, developmental assessment

Answer 710

Doctors Physiotherapists – to help with the tone and posture problems SALT – to help with swallowing problems OT – if any adjustments at home needed Child and parents School – especially the special educational needs team

Answer 711

Baclofen – oral or intra-thecal Oral diazepam Botox injection Orthopaedic surgery

Answer 712

It’s an accurate representation of brain size and development Microcephaly – can indicate the brain has not formed properly, particularly if it’s sloped at the front, and could mean they will have learning difficulties Macrocephaly – can indicate a child has hydrocephalus or SOL BEWARE – small babies will have small heads!! Compare to their centile for weight and length. If they are on 2nd centile for everything they are just small. If they are on 6th for weight and length and 2nd for head, their head is too small

Answer 713

0.45% sodium chloride + 5% dextrose 0.9% sodium chloride + 5% dextrose

Answer 714

100, 50, 20 100 mls/kg/day for the first 10kg 50 mls/kg/day for the next 10kg (so up to 20kg) 20 mls/kg/day for every kg after

Answer 715

(100 x 10) + (50 x 10) + (20 x 3) = 1560 ml/24 hours (1 day) So hourly rate = 1560/24 = 65ml/hour

Answer 716

10% dextrose only Do not need saline on day 1

Answer 717

Hypoglycaemia Hypovolaemia (shock)

Answer 718

10% dextrose 2mls/kg stat

Answer 719

15 x 2 = 30 30mls bolus of 10% dextrose

Answer 720

NORMAL SALINE 0.9% (even in newborn babies) 20mls/kg stat

Answer 721

Correction of dehydration = % dehydration x 10 x weight in kg

Answer 722

Parental mental health problem Low socioeconomic status/deprivation Parental learning difficulty Alcohol and substance misuse “Difficult child” Disabled child Preterm infants Born to substance misusers

Answer 723

History that does not fit with the visible injuries: Too many injuries Wrong site Unusual shape or pattern Wrong type of incident Delay in presenting to hospital (old injuries) No history A history that changes

Answer 724

Shins – external bony bits Knees Elbows Toddlers – bumps on heads from walking into things

Answer 725

Abdomen Genitalia Insides of arms and legs (bits that are tucked away) Behind the neck/any other soft bits Young babies that cannot roll over or move yet should not have any bruises at all – massive red flag. RIB FRACTURES IN BABIES also very specific for NAI

Answer 726

Subdural haematoma

Answer 727

A standard of care that does not meet the needs of a child Types – food and drink, warmth, education, emotional support

Answer 728

Medical assessment Lab tests – FBC and platelets if they are prone to clotting Swabs – skin sores (could be impetigo etc.) Bone profile if fractures (calcium, vit D, PTH) Skeletal survey Developmental assessment Social services assessment

Answer 729

Miserable Pyrexia Temperture Vomiting E.Coli Treatment – IV cefuroxime and USS urinary tract system

Answer 730

By producing beta lactamase Breaks down the beta lactam part of the antibiotic making it uneffective

Answer 731

Public Health England

Answer 732

Empyema Will never resolve by antibiotics Needs surgical drainage

Answer 733

Potential to cause bowel obstruction or perforation Hernia – cannot get above it in examination Hydrocele – you can get above it

Answer 734

Thyroglossal – midline, smooth, moves when they stick their tongue out Branchial – NOT in the midline, tend to appear along the border of the sternocleidomastoid Dermoids – lateral aspect of the eye, produce sebaceous material. Beware because can communicate intracranially and cause meningitis

Answer 735

Bowel obstruction Either due to intussusception, malrotation, volvulus etc

Answer 736

Intussusception

Answer 737

Congenital diaphragmatic hernia

Paediatric FC from ppt Flashcards

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