PAEDS gastro, renal, MSK, endocrine, neonates Flashcards
1
Q
What are the clinical features of IBS?
A
Abdominal pain, often worse before defecation
Bloating
Explosive, loose or mucousy stools
Feeling of incomplete defecation
Constipaton - often alternating with normal or loose stools
2
Q
When should H.pylori or duodenal ulcers be suspected? How would you test for this and treat it?
A
If epiastric pain that keeps them awake at night or history of peptic ulcers in first degree relative
H.pylori causes nodular antral gastritis - identified on antral biopsies
C-labelled urea by mouth and C13 breath test
Treat - amoxicillin + metronidazole/clarithromycin and PPI
3
Q
What is the commonest cause of gastroenteritis in the developed countries?
A
Rotavirus
Presents winter to spring in under 2 yrs
4
Q
What features of gastroenteritis suggest bacterial infection? Give some examples of causative organisms
A
Blood in stools Campylobacter jejuni Shigella Cholera E.coli
5
Q
Give the features of gastroenteritis
A
Sudden watery or loose stools often with vomiting
Contact or recent travel abroad
6
Q
What assessment must be done in a child with gastroenteritis?
A
Hydration!
No clinical dehyration (<5%)
Clinical dehydraton 5-10%
shock >10%
7
Q
How does hyponatraemic dehydration occur?
A
Sodium and water leave in diarrhoea together so plasma concentration stays the same. But when drink hypotonic solution/water, they retain water but lose sodium causing hyponatraemia.
Causes shift of water into ICF - increased brain volume, convulsions
8
Q
How does hypernatraemic dehydration occur?
A
Somtimes water loss exceeds sodium loss - so plasma sodium conc increases
Usually in fever, hot, dry environment or low-sodium diarrhoea
Causes shift from ICF to ECF of water - sunken fontanelle, eyes, reduced tissue elasticity
Cerebral shrinkage - jittery movements, increased muscle tone, hyperreflexia, altered consciousness, seizures, cerebral haemorrhage (small)
Transient hyperglycaemia
9
Q
What investigations would you do in gastroenteritis?
A
Usually none needed. Stool culture if child is septic, blood or mucus or immunocompromised
Check electrolytes, urea, creatinine, glucose
Blood culture if going to start antibiotics
10
Q
How would you manage hypernatraemic dehydration?
A
Oral rehydration solution
IV fluids slow (<0.5mmol/L per hour) over 48hrs, regular monitoring of sodium (risk of hyponatraemic cerebral oedema)
11
Q
When would you use antibiotics in gastroenteritis?
A
Not routinely even if bacterial
Only if sepsis, extra-intestinal spread of infection, salmonella gastroenteritis <6 months, malnourished or immunocompromised children or specific ones
(C. diff, cholera, shigellosis, giardiasis)
12
Q
How would you support a child nutritionally after gastroenteritis?
A
Increased nutritional intake
Zinc supplementation
13
Q
What is post-gastroenteritis syndrome?
A
Return to normal diet after gastroenteritis can result in watery diarrhoea
Transient lactose intolerance (Clinitest positive - non-absorbed sugar in stools)
Return to oral rehydration solution for 24hrs, then re-introduce normal diet
Other allergies may occur e.g. CMPA, coeliac - may need specialist diet or parenteral nutrition until mucosa recovers
14
Q
When and how does coeliac disease present?
A
8-24mnths when introduce wheat foods Failure to thrive Abdominal distension Buttock wasting Abnormal stools General irritability
15
Q
What are some features of Crohn’s disease?
A
Failure to thrive, delayed puberty, abdo pain, diarrhoea, weight loss, fever, lethargy, oral or perianal lesions, arthralgia, uveitis, erythema nodosum
Raised ESR, CRP, platelet count
Iron deficiency anaemia, low serum albumin
Transmural, subacute, chronic - mostly affecting distal ileum and proximal colon
Inflammation, then strictures and/or fistulae
16
Q
How is Crohn’s disease diagnosed?
A
Upper and lower GI endoscopy biopsies
Non-caseating epithelioid cell granulomata
Imaging - narrowing, fisuring, mucosal irregularities, bowel wall thickening
17
Q
How would you induce and maintain remission in Crohn’s?
A
Normal diet replaced by whole protein modular feeds (polymeric diet) for 6-8 wks. Systemic steroids if ineffective
If relapse: Azathioprine, mecaptopurine or methotrexate to maintain remission
Infliximab/adalimumab if these fail
Surgery for complications
18
Q
How does ulcerative colitis present?
A
Rectal bleeding, diarrhoea, colicky pain
Weight loss, failure to thrive (less than Crohn’s)
Erythema nodosum, arthritis
19
Q
How would you diagnose ulcerative colitis?
A
Upper and lower endoscopy - exclude infective causes of colitis, check small bowel for crohns
Histology - mucosal inflammation, crypt damage, ulceration
20
Q
How is ulcerative colitis managed?
A
Mild - aminosalicylates (balsalazide, mesalazine)
Possibly topical steroids or systemic if more aggressive
Fulminating disease - IV fluids, steroids
Ciclosporin if this fails to induce remission
Colectomy with ileostomy if severe/toxic megacolon
Regular colonoscopic screening after 10yrs from diagnosis
21
Q
What is definition of constipation?
A
Infrequent passage of dry, hardened faeces often accompanied by straining or pain
22
Q
What conditions should be considered in a child with constipation?
A
Baby - Hirschsprung disease, anorectal abnormalities, hypothyroidism, hypercalcaemia
Dehydration, toilet training, unpleasant toilets or stress
23
Q
What does examination show in constipation?
A
Palpable mass in abdomen, in well-looking child
PR only if pathology suspected by specialist
24
Q
What can occur in long-standing constipation?
A
Rectum becomes overdistended, loss of feeling the need to defecate and involuntary soiling due to overflow
25
How would you manage acute or chronic constipation?
Acute - mild laxatives and extra fluids
Chronic with overflow - Disimpaction regimen - stool softeners with macrogol laxative (e.g. Movicol) with escalating dose over 1-2wks. If not tolerated, osmotic laxative
If unsuccessful - stimulant (senna, sodium picosulphate) as well
Maintenance - macrogol (+stimulant) reduced over months
If severe impaction - enema or manual evacuation under anaesthetic
26
What is Hirschsprung disease?
Aganglionic section of large bowel - narrow, contracted segment from rectum proximally (distance varies) - mostly rectosigmoid
Causes dilated colon at normal innervation sites
27
How does Hirschsprung disease present?
Neonatal - failure to pass meconium in first 24hrs
Abdominal distension and bile-stained vomiting
PR - narrowed segment with liquid stool and flatus on withdrawing of finger
Sometimes infants present due to C.diff infection with Hirschsprung enterocolitis
Later childhood - chronic constipation, abdo distension, no soiling, growth failure
28
How is Hirschsprung disease diagnosed and managed?
Rectal biopsy - absence ganglion cells and large acetylcholinesterase positive nerve trunks
Anorectal manometry or barium studies - length of section
Management - surgery - colostomy, anastamose innervated bowel to anus
29
What tests may assess renal function in children?
Plasma creatinine concentration
eGFR - better as does not depend upon muscle bulk like creatinine does
inulin or EDTA GFR - more accurate, but repeated blood tests so not used as much in children
Creatinine clearance - requires timed urine collection and blood tests so not used as much
Plasma urea concentration - increased in renal failure before creatinine Also in high protein or catabolic state
30
Why might congenital kidney disease not be obvious for the first few days of life?
Newborn kidney has low GFR and urine flow so signs of obstruction may not be obvious especially if mild, until urine flow demand increases
31
How might a UTI present?
Varies - subtle as food aversion and severe as sepsis
Unexplained fever >38
If alternative site of infection, but does not improve when treated for other site of infection
If older - abdominal pain or dysuria
Vomiting common
32
Which children should ALWAYS get urine microscopy and culture if presenting with fever?
Infants under 3 months!
33
How might urine samples be obtained in children?
If able to wee on command - MSU
Otherwise "clean catch"
Also collection pads/adhesive plastic bag - less sterile
Catheter or suprapubic aspiration
Always say how urine sample collected on form
34
What might be done with urine sample to check if UTI?
Look at urine first
Urinalysis - nitrites, leukocytes
MC+S - what organism, what antibiotics
35
What do urinalysis and MC+ results suggest for treatment?
Positive WCC and growth = UTI, treat Abx
if growth - treat as UTI regardless of WCC
Positive WCC and no growth - treat with antibiotics if symptomatic
36
What is the bacteriological criterion for UTI?
More than 100 000 (10 to power 5) organisms per ml of urine on CCU/MSU
OR any growth on suprapubic aspiration
37
What organisms cause UTIs in children?
Commonest = E. coli
Proteus also commoner in boys
if abnormal organism e.g. Pseudomonas - suspect structural abnormality
38
What 3 groups require admission to hospital for suspected UTI?
```
All children <3 months
Systemically unwell (fever, loin pain, tenderness)
Risk factors (kidney transplant, renal disease)
```
39
What treatment is given for children systemically unwell with suspected UTI?
MIN 2 days IV antibiotics (cefotaxime then adjusted), send home on 7-10 days oral
Advise to return if no better at 24-48hrs
40
What treatment would be given to children with suspected UTI <3 months and systemically well?
2 days IV Abx (cefotaxime then adjusted) and 3 days oral
| Advise to return if no better at 24-48hrs
41
Who requires follow up after UTI to check for structural abnormalities?
Recurrent or atypical UTIs
42
What defines an atypical UTI?
```
Septicaemia/requires IV antibiotics
Non E.coli UTI
Poor urine flow
Abdominal mass/bladder mass
Raised creatinine
Failure to respond to suitable antibiotics within 48hrs
```
43
What defines a recurrent UTI?
2 or more UTI episodes with at least one episode of systemic signs/symptoms
3 or more UTI episodes without systemic signs/symptoms
44
How would you investigate for structural abnormalities in urinary tract?
USS urinary tract - observer dependent, dynamic, allows size of kidneys, bladder and drainage checked
If abnormality present or <3yrs: (wait 3 mnths if poss)
Micturating Cystourethrogram (MCUG) - urinary catheter into bladder, fills it with dye to see if vesicoureteric reflex, gives outline of bladder and urethral valve (<3yrs)
DMSA - renal scarring, relative renal function (IV cannula giving nuclear dye taken up by kidney) - 1-3yrs
45
Other than antibiotics what supportive measures are done to prevent UTIs?
```
High fluid intake
Regular voiding, double micturition
Prevention or treatment constipation
Good perineal hygiene
Probiotic - lactobacillus acidophilus
Antibiotic prophylaxis? - under 2yrs with urinary tract abnormality or severe reflux (trimethoprim at night)
```
46
How should children with recurrent UTIs, renal scarring or reflux be followed up?
Urine culture with any non-specific illness
Long-term low dose antibiotic prophylaxis
Circumcision in boys
Anti-reflux surgery - if progresses, but unsure
Blood pressure checked annually, height, weight, test for proteinuria
Regular assessment of renal growth and function
Further symptomatic UTIs - investigate to see if new scars or continuing reflux
47
What different ways can oedema present?
```
Facial puffiness
Pitting oedema
Pulmonary oedema
Ascites
Pleural effusions
```
48
What is the triad of symptoms for nephrotic syndrome?
Heavy proteinuria
Hypoalbuminaemia
Oedema - periorbita, scrotal/vulval, legs/ankles, ascites, breathlessness
49
What tests on urine sample are indicative of nephrotic syndrome?
Urine looks frothy/foamy
3+ protein or more on dipstick
But best is:
First morning Urine Protein:Creatinine Ratio (normal <20, hypoalbuinaemia>600)
50
How might you test for hypoalbuminaemia?
Blood test: albumin.
| Normal = 35-45, hypoalbuminaemia <25-30
51
Why are nephrotic patients at increased risk of infection or thrombosis?
Loss of protein through kidneys/urine occurs with antibodies and anti-thrombotic factors too
52
When might proteinuria occur in children?
```
Transient proteinuria in febrile illness/after exercise
Orthostatic proteinuria
Glomerular abnormalities
Increased glomerular filtration pressure
Hypertension
Reduced renal mass
Tubular proteinuria
Nephrotic syndrome
```
53
Give features of steroid-sensitive nephrotic syndrome
```
Age 1-10 yrs, commoner in Asian children, weak association atopy
Normal blood ressure
No macroscopic haematuria
Normal complement levels
Normal renal function
Minimal change on biopsy
Responds to steroids
```
54
How would you treat steroid-sensitive nephrotic syndrome?
4 weeks ORAL PREDNISOLONE 60mg/m2
then
4 weeks at 40mg/m2 on alternate days
Other meds: Water and sodium moderation, diuretics
Penicillin, vaccines for measles, VZV, pneumococcus
55
What are the features of steroid-resistant nephrotic syndrome?
```
Raised BP
Haematuria
Impaired renal function
often similar to nephritis
Failure to respond to steroids
Histology - glomerulopathy - basement membrane abnormality
```
56
How is steroid-resistant nephrotic syndrome treated?
Referred to paediatric nephrologist
| Oedema managed - diuretics, salt restriction, ACE-I, sometimes NSAIDs which reduce proteinuria
57
What are features of congenital nephrotic syndrome?
presents in first 3 months of life
Most common is recessive
High mortality rate due to hypoalbuminaemia
Unilateral nephrectomy may be required to stop proteinuria, followed by dialysis for renal failure until can get renal transplant
58
What can cause nephrotic syndrome?
```
Congenital
Many are unknown in cause
HSP and other vasculitides
SLE
Infections
Allergens
```
59
What is minimal change disease?
Cause of nephrotic syndrome - glomerular disease but no change seen on microscope
Requires electron microscope to see pathology
Symptoms of nephrotic syndrome may occur quicker
60
How do you determine haematuria?
Urine dipstick - if Hb on dipstick should be done under microscopy
>10 RBC per high power field
61
How might urine from glomerular haematuria present?
Brown urine - RBC deformed as pass through glomerular membrane and casts
62
What are the features of glomerulonephritis?
Visible haematuria, some proteinuria
Impaired GFR, raised creatinine, less urine output
Salt and water retention - oedema, HTN
63
What is the most common cause of glomerulonephritis?
Post-streptococcal nephritis (Group A B haemolytic strep) - body autoimmune response to antigens on kidneys similar to those on bacteria
Nephritis usually 10 days post-infection
64
How is post-streptococcal glomerulonephritis diagnosed?
Evidence of recent strep infection (culture of organism from throat swab, raised ASO/antiDNAse B titres)
Low complement C3 + C4 levels (normal in 3-4wks)
65
How is glomerulonephritis managed?
```
Fluid balance
Reduce fluid intake
Diuretics, salt restriction
Correct other electrolyte imbalances
Dialysis may be needed
Penicillin - to correct strep infection
```
66
What is HSP?
Vasculitis affecting multiple parts of body
Presents with: (often fever)
Skin rash - leg extensors, buttocks, non-blanching purpura raised and palpable
Arthralgia, periarticular oedema - red, swollen
Abdominal pain - petechiae, bleed, intussusception
Glomerulonephritis
Biopsy - shows IgA deposition but diagnosis made off clinical assessment of rash
67
What may occur in the kidneys as a result of HSP?
```
Asymptomatic
Nephrotic syndrome
Glomerulonephritis/acute nephritis
Haematuria/proteinuria
Renal impairment
Hypertension
```
68
How is HSP managed?
Analgesia, watch and wait
Corticosteroids if severe colicky abdo pain
FBC, U+Es, CPR, ESR
Urinalysis - for degree of haematuria/proteinuria. If heavy proteinuria - renal biopsy to decide treatment
Prednisolone if nephrotic syndrome occurs
69
What syndrome is familial nephritis?
Alport syndrome - X-linked recessive
End-stage renal failure in early adult life in males
Sensorineural deafness and ocular defects
Mother may have haematuria
70
What types of vasculitis other than HSP can cause nephritis?
```
Polyarteritis nodosa
Microscopic polyarteritis
Wegener granulomatosis
Check for ANCA
Treat - steroids, plasma exchange, IV cyclophosphamide
```
71
What should always be investigated if there is haematuria?
All regardless: Urine microscopy
Protein and calcium excretion
Kidney and urinary tract USS
Urea, electrolytes, creatinine, calcium, phosphate, albumin
FBC, platelets, clotting screen, sickle cell screen
Glomerular haematuria:
ESR, complement, anti-DNA
Throat swab, antistreptolysinO/anti-DNAse
Hep B and C screen
Renal biopsy if indicated
Test mother's urine for blood and hearing test (Alport)
72
What might suggest nephritis due to SLE?
Multiple antibodies - anti-dsDNA, ANA
Low C3 and C4
Other symptoms:
Malaise, arthralgia, malar rash (often photosensitive)
73
What are some congenital renal malformations?
```
Multicystic dysplastic kidney
Autosomal recessive PCKD, autosomal dominant PCKD
Tuberous sclerosis
Pelvic kidney or horseshoe kidney
Duplex system
Bladder extrophy
Prune-belly syndrome (absent muscular syndrome)
Neuropathic bladder
Posterior urethral valves
```
74
What is vesicoureteric reflux?
Developmental anomaly of vesicoureteric junctions
Familial or with neuropathic bladder, urethral obstruction, temporarily after UTI
Ureters displaced laterally, at less of an angle and shorter intramural course
Reflux amount varies
Intra-renal reflux increases risk of renal scarring if UTIs occur. Increased risk pyelonephritis, UTI, renal damage due to bladder voiding pressure
75
What is the triad of haemolytic uraemic syndrome?
Acute renal failure
Microangiopathic haemolytic anaemmia
Thrombocytopenia
76
What is HUS often secondary to? Give pathophysiology of HUS
Gastrointestinal infection e.g. E.coli O157:H7 (animal contact or meat) or Shigella
Prodrome of bloody diarrhoea
Toxin enters GI mucosa and localises to kidney endothelium - causes intravascular thrombogenesis and activation of coagulation cascade - normal clotting
Platelets consumed by this and damage to RBC in microcirculation which is occluded
Brain, pancreas and heart may also be affected
77
How is HUS managed?
Supportive therapy, including dialysis (good prognosis)
Follow-up to check for persistent proteinuria, hypertension, renal decline in function
Intracerebral involvement - plasma exchange or plasma infusions
78
What is atypical HUS?
No diarrhoeal prodrome
May be familial and frequent relapses
High risk of HTN and mortality
Also may need plasma exchange or plasma infusions
79
What are the commonest causes of acute renal failure in children?
Pre-renal - hypovolaemia, circulatory failure
Renal - HUS or multi-system failure
Post-renal - urinary tract obstruction
Manage - treat underlying cause, metabolic abnormalities, dialysis if necessary
80
What are features of nocturnal enuresis?
Bed wetting at night, boys:girls is 2:1
Genetically determined delay in acquiring sphincter competence - 2/3 first degree relative
Interference in learning to become dry - stress or lack of parental approval
Organic cause: UTI, faecal retention pressing on bladder, osmotic diuresis (diabetes, chronic renal failure)
81
What investigation should be done in nocturnal enuresis?
Urinalysis - glucose, protein, check for infection
82
How is nocturnal enuresis managed?
Treatment rarely taken until after 6yrs old
Instead, explain that is common, beyond conscious control and stop any punishments
Star chart - reward for dry bed
Enuresis alarm - sensor on bed or child's pants and sounds when bed wet, wakes child to go and pass urine
Desmopressin - short term relief for bed wetting e.g. holidays, sleepovers
Self-help groups
83
When are fractures more common in children?
More common in boys
More common when bone growth rate at its fastest
Very common in mobile children >1yr. Be wary if non-mobile and fracture - NAI?
84
Give WHO definition of osteoporosis
Low bone mass and microarchitectural deterioration of bone tissue leading to enhanced bone fragility and consequent increased risk of fracture
85
What may suggest osteoporisis in children?
1 or more vertebral crush fractures
2 or more long bone fractures by age 10
3 or more long bone fractures by age 19
Bone density scan - < - 2SD
86
What are the possible causes of acquired osteoporosis?
```
Drug-induced (especially steroids)
Endocrinopathies - e.g. raised corticosteroids
Malabsorption
Immobilisation
Inflammation
```
87
What are the inherited causes of osteoporosis?
```
Commonest = osteogenesis imperfecta
Others = galactosemia, haematological disorders, idiopathic
```
88
What is the commonest inheritance pattern and genetic pathology behind osteogenesis imperfecta?
Mostly autosomal DOMINANT
Defect in gene coding for type 1 collagen
Causes bones to be FRAGILE and BRITTLE
89
What are some presenting features of osteogenesis imperfecta?
Fractures, Deformities due to fractures or bowing deformities (type 1), Bone pain
Impaired mobility, Poor growth
Blue appearance to sclerae
Deafness
Hernias
Valvular prolapse
The more severe, the more growth is affected
90
What are the different types of OI?
```
1 = mild
2 = lethal
3 = progressively deforming, severe
4 = moderate
```
91
What occurs in type 2 OI?
Many infants are stillborn or born with multiple fractures
| Lethal - no air in lungs and major deformities
92
How is OI managed?
MDT - physician, surgeon, dietitian, physio, nurse
BISPHOSPHONATES
Surgery - may have rods to repair deformities etc.
93
How is osteoporosis managed?
Dietary supplements - calcium, vit D
Regular weight-bearing exercise
Minimise corticosteroids
Sometimes Bisphosphonates
94
What is rickets?
Decreased mineralisation of bone
| often due to vitamin D or calcium deficiency
95
What features may be present in history of a child with rickets?
```
Poor sunlight exposure
Low vitamin D dietary intake
Darker skin
Swollen costo-chondral joints
Limb deformity, bow legs, metaphyseal swellings
Weakness
Misery, bone pain
Respiratory distress
```
96
What is the role of vitamin D/calcitriol?
- increases calcium absorption from gut and calcium resorption from bone, increase renal calcium reabsorption from PCT
- immune function
- maternal vit D influences bone size and mass in childhood
97
What occurs in large vitamin D deficiencies in babies who are exclusively breastfed?
```
Life-threatening cardiomyopathy
Hypocalcaemia
Convulsions
Rickets
Bowed legs when start walking
Often have gross motor delay
```
98
What other organ dysfunction may be leading to vitamin D deficiency?
Kidney disease - produces active vitamin D
Liver disease - produces precursor to vit D
Also biliary disease - lack of bile prevents absorption of fab-soluble vitamin D e.g. cholestasis, biliary atresia
99
How is rickets treated?
Treat calcium deficiency as hypocalcaemia can be dangerous
Vitamin D: <2yrs 3000 units/day, 2-10yrs 6000 units/day, >10 yrs is 10 000 units/day
Stoss therapy if complicance issue - high dose vit D stat
100
What are the commoner causes of limp in children aged less than 3 years?
Trauma, Infection (cellulitis, osteomyelitis), developmental dysplasia of the hip
101
What are the commoner causes of limp in children aged 3-10yrs?
```
Trauma
Transient synovitis
Osteomyelitis
Septic arthritis
Perthe's disease
```
102
What are the commoner causes of limp in chidlren over 10yrs old?
Trauma, OA, septic cellulitis
Slipped upper femoral epiphysis
Anterior knee pain (chondromalacia)
Perthe's
103
Where is pain commonly referred to in children with limp?
Hip pain often referred to knee!
| If child complains of knee pain, always check hip
104
What other area outside of limbs and spine is important to examine for other causes?
Abdomen - appendicits and constipation can cause limp. Testicles may also cause limp
105
What is Toddler's fracture?
undisplaced spiral fracture of tibia in pre-school children
Usually only small fall or twisting injury
Tenderness over tibial shaft, may not see fracture on XR
Immobilise for management
106
What is transient synovitis/irritable hip?
Inflammation of synovium - commonest cause of hip pain in children aged 2-12 yrs
Often follows or accompanied by resp viral infection
affects boys more, usually unilateral
Sudden onset pain in hip or knee, limp, refuse to walk
No pain at rest or passive movement
Decreased ROM
107
How would you investigate transient synovitis?
XR - may be normal
USS - may show effusion
FBC and ESR slightly elevated or normal
108
How would you manage transient synovitis?
Bed rest
| Symptomatic + pain relief - ibuprofen
109
What is the most common form of arthritis in childhood?
Reactive arthritis
- transient joint swelling <6 wks often ankles/knees
- Usually follows gastroenteritis, STIs, Lyme disease, rheumatic fever, post-streptococcal
Low grade fever, mildly elevated CRP, WCC
XR normal
No treatment, NSAIDs pain relief
110
Give features of septic arthritis
Usually <2 yrs but any age possible
Unwell, non-specific early signs, fever
Pain at rest, resist attempt at movement, reluctant to weight bear, ROM reduced and painful
Red, hot, swollen joint acutely. Raised WCC, CRP
XR - may be normal or effusion (if later)
USS - effusion
Can cause permanent damage to peri-articular bone
111
How is septic arthritis managed?
FBC, CRP, ESR
Blood cultures
USS of deep joints, XR to exclude trauma/others
MRI - to exclude adjacent osteomyelitis?
Immediately joint aspiration under US - culture
Then give antibiotics - prolonged IV (ceftriaxone)
Initially immobilised then mobilised
May require surgical drainage
112
What is Knocher's criteria?
Criteria used to determine likelihood of being septic arthritis using fever, weight bear, ESR in first hour, WBC more than 12
Fever and CRP are most important
113
What is Perthe's disease?
Avascular necrosis of the capital femoral epiphysis
Followed by revascularisation and reossification over 18-36 months
Mainly affects boys aged 5-10yrs
114
How does Perthe's disease present?
Insidious, onset of limp or hip or knee pain, mostly unilateral but can be bilateral
Similar in presentation to transient synovitis
Movement of hip painful especially on internal rotation
No history of trauma
115
What might be shown on XR for Perthe's disease?
Might do bilateral if suspect
May be no signs or sclerosis, fragmentation and irregular femoral head
Bone scan or MRI may be helpful if no changes seen on XR to make diagnosis
116
How is Perthe's disease managed?
Less than half femoral head affected or under 8 - bed rest and traction
More than half or over 8 - plaster or calipers
Or surgery - femoral or pelvic osteotomy
Older children more likely to go on to develop degenerative arthritis in adult life, but young children recover well
117
What is a SUFE?
Slipped upper femoral epiphysis
Posteroinferior displacement femoral epiphysis - needing prompt treatment to prevent avascular necrosis.
Most common at 10-15 yrs during growth spurt
Commonest in obese boys, bilateral in 20%, AfroCaribbean
Associated with metabolic abnorms e.g. hypothyroidism, hypogonadism
118
How does SUFE present?
Hip, thigh or knee pain, limp
| Possible leg length discrepancy or external rotation of limb
119
What would an XR show for SUFE?
XR diagnosis AP and request frog lateral view
Widening and irregularity of epiphysis
Posteroinferior displacement epiphysis
120
How is SUFE managed?
Pin fixation in situ (with/without abduction)
121
What is osteomyelitis? Where are the most common sites? What pathogens cause the infections?
Infection of metaphysis of long bones
Distal femur and proximal tibia commonest
Can lead to septic arthritis if joint capsule distal to
epiphysis
Pathogens: S. aureua, Strep, Hib, TB (rare), salmonella in SCD
122
How does osteomyelitis present?
Acute onset painful, immobile limb - extreme pain on movement
Acute febrile illness
Swelling, tenderness, red, warm joint
May be sterile effusion of adjacent joint
Can be back or groin pain if vertebral/pelviic infection.
Infection can be disseminated in staph or Hib
123
What investigations would you do for osteomyelitis?
Blood cultures, FBC, CRP - positive
XR - initially normal, soft tissue swelling (takes 7-10 days for new bone formation and rarefaction to see)
US - may show periosteal elevation at presentation
MRI!!! - allows identification of infection in bone - subperiosteal pus, purulent debris in bone
Radionuclide bone scan - if site unclear
Bone biopsy???
124
How would you treat osteomyelitis?
Prompt IV antibiotics (anti-staph vancomycin, clindamycin, nafcillin), then switch to oral
Take for several weeks - prevent necrosis, chronic infection, limb deformity, amyloidosis
Rested in splint, then mobilised later
If atypical or immunodeficiency - aspiration or surgical decompression of periosteal space
Surgical drainage if does not respond quickly to treatment
125
What malignancies may present with limb pain?
ALL - bone pain primarily at night, possible arthritis
Neuroblastoma - systemic arthritis, bone pain from metastases
Bone tumours - osteogenic sarcoma, Ewing tumour (pain, swelling, pathological fracture)
Osteoid sarcoma (benign) - adolescent boys, night bone pain improving with NSAIDs, soft tissue swelling, tender,
126
What would XR show for osteoid sarcoma? How would you manage??
XR - radiolucent nidus of osteoid tissue surrounded by sclerotic bone
If normal - do CT or MRI
Treatment - surgical removal
127
What is Osgood-Schlatters disease?
Osteochondritis of patellar tendon insertion at the knee, often adolescent males who are active
Knee pain after exercise, localised tenderness, swelling over tibial tuberosity
Often hamstring tightness, bilateral 25-50%
Resolves with reduced activity, physio for quadriceps strengthening, hamstring stretches, orthotics
Knee immobiliser splint may help
128
What are the features of JIA?
Persistent joint swelling >6 weeks
often presents before age 16yrs, absence of infection
Classified into subtypes by oligo, poly, systemic, rheumatoid factor, HLA b27
Stiffness after resting (seizing up), intermittent limp, deterioration of behaviour and mood
May only be minimal joint swelling or lots in chronic
Leg lengthening, valgus deformity, digit discrepancy
Systemic - fever, SALMON PINK RASH, anterior uveitis, flexion contractures, growth failure
129
What are complications of JIA?
Chronic anterior uveitis
Flexion contractures of joints
Growth failure - anorexia, chronic disease, systemic corticosteroids
Anaemia of chronic disease, delayed puberty
Osteoporosis
Amyloidosis - proteinuria, renal failure, high mortality
130
How is JIA managed?
```
MDT - regular ophthalmology screening, physio, dentistry, orthopaedics
NSAIDs and analgesics
Intra-articular injections
Methotrexate
Systemic corticosteroids - if necessary
Cytokine modulators, biologics
```
131
What is the prognosis of JIA?
At least 1/3 will have adult disease due to joint damage or visual impairment or fractures from osteoporosis, psychosocial issues
Long-term outcomes are improving
132
What two manouevres are done in the newborn and infant physical examination to check for DDH?
Barlow - check if hip can be dislocated posteriorly out of acetabulum
Ortolani - Can be relocated back into acetabulum on abduction
133
Why is early detection of DDH important?
Early detection - DDH usually responds to conservative management
Late - hip dysplasia, requires complex treatment including surgery
134
How might DDH present?
Neonatal screening
After this - abnormal gait or limp, asymmetry of skin folds around the hip, limited abduction of the hip or shortening of affected leg`
OA of hip and back, pain
135
What are the risk factors for DDH?
Family history
Breech presentation at 36 weeks gestation or more
Breech delivery
Multiple pregnancy
136
How should children be referred from the newborn and infant screening programme with possible DDH?
NEWBORN
Abnormal findings - Refer for USS and expert opinion within 2 WEEKS
Normal but FH - Refer for USS and expert opinion within 6 WEEKS
INFANT - abnormal exam - refer for USS and expert opinion within 10 WEEKS
137
How is DDH managed?
If USS abnormal and early - splint or harness to keep hip flexed and abducted for several months
Monitor progress by USS
Risk of necrosis of femoral head if done badly!
Surgery required if conservative measures fail or too late
138
What type of rash forms in Stevens-Johnson syndrome?
Severe bullous form of erythema multiforme involving mucous membranes
139
What other features may occur in Stevens-Johnson syndrome?
Conjunctivitis, corneal ulceration, uveitis
Ophthalmological assessment required
May be caused by infection or drug sensitivity
Infection, toxaemia, renal damage - possibly fatal
140
What are the core features of diabetes?
Toilet - polyuria, secondary/nocturanl enuresis
Thirsty
Tired
Thinner
(children mostly polydipsia and weight loss)
Urine - glucose and proteins in urine
141
What levels of blood glucose are diagnostic of diabetes in random, fasting and OGTT?
diagnostic: random: >11.1 + symptoms or twice and asymptomatic
if unsure - fasting: >7 or HbA1c raised
rare - OGTT: >11.1 (2hrs after 75g oral glucose)
142
When does diabetes commonly present?
Pre-school children
Teenagers
Spring and autumn
143
What features may suggest type 2 instead ot type 1 diabetes?
Family history
Children from Indian subcontinent
Severely obese children
Signs of insulin resistance - acanthosis nigricans, skin tags, polycystic ovary signs
144
What are signs of diabetic ketoacidosis? What is diagnostic of DKA?
Signs: acetone breath, vomiting, dehydration, abdo pain, hyperventilation due to acidosis, hypovolaemic shock, drowsiness, coma, death
diagnostic - glucose>11.1 and ketones>3.0 in blood
145
Give the pathophysiology of DKA
Absence insulin - hyperglycaemia - starvation of cells so metabolise fats - ketones - acidosis
osmotic diuresis - lose water, hypovolaemia
Can lead to cerebral oedema and shock
146
What essential investigations should you do in suspeted DKA?
Blood glucose, blood ketones
U+E, creatinine (dehydration)
Blood gas analysis (severe metabolic acidosis)
Urinary glucose and ketones
Ask for evidence of precipitating cause e.g. infection - do blood and urine cultures
Cardiac monitor - T wave changes in hypokalaemia
Weight
147
How would you manage a DKA?
Fluids - resuscitaton if needed + dehydration and maintenance over 48hrs - avoid rapid rehydration as can cause cerebral oedema
Insulin - start after 1hr, titrate according to blood glucose, switch to 4% dextrose after 24hr to avoid hypoglycaemia
Potassium - can fall due to insulin - give as soon as urine is passed - cardiac monitoring, monitor electrolytes including potassium, ketones every 2hrs
Fluid balance
Switch to SC insulin (stop IV 1hr after)
Antibiotics if fever - DKA no fever so could be infection
148
What blood pH/bicarbonate indicates mild DKA? What fluid deficit is assumed for this?
pH 7.2-7.29 or bicarbonate <15
| Assume 5% fluid deficit
149
What blood pH/bicarbonate indicates moderate DKA? What fluid deficit is assumed for this?
pH 7.1-7.19 or bicarbonate <10
| Assume 7% fluid deficit
150
What blood pH/bicarbonate indicates severe DKA? What flulid deficit is assumed for this?
pH <7.1 or bicarbonate <5
| Assume 10% fluid deficit
151
What bolus is given to all non-shocked patients with DKA? What must be done with this in the calculations?
10ml/kg bolus for non-shocked
| Should subtract this fluid from hydration+maintenance fluids when calculating 48hr fluids
152
What is the formula for fluid requirement over 48hrs in DKA?
Shocked: Fluid requirement = Deficit/48 + maintenance/48
Non-shocked: = (Deficit-bolus)/48 + maintenance/48
For both maintenance and deficit, if overweight or obese, use max 80kg or 97th centile
153
What is the Holiday-Segar forumular for calculating maintenance fluids?
100ml for first 10kg
50ml for second 10kg
20ml for each kg over 20kg
154
How might insulin be given in diabetes?
Rapid, intermediate and short-acting. Mixtures of all 3 may also be used
Mostly - insulin pump OR
3-4 times a day injection regimen (basal bolus) - short acting before each meal/snack and long-acting late evening or before breakfast
155
What diet modifications may be made in diabetes?
Complex carbohydrates and fibre good
Low fat content
Carbohydrate counting - calculate insulin requirements based on BM and demands e.g. exercise
156
What are good limits for BM for diabetic children and adolescents?
```
Want to avoid hypos!
Children : 4-10mmol/L
Adolescents: 4-8mmol/L
Want 4 or more tests a day in diary
OR continuous glucose monitoring sensors
Check HbA1C 3x a year
```
157
What should always be checked if a diabetic child has an infection?
Blood ketones - incase of DKA
158
What are some symptoms of hypoglycaemia? How is it managed?
```
Hunger, tummy ache
Sweatiness
Faint, dizzy, "wobbly"
Pallor, irritable
Neuroglycopenic - confusion, drowsy, visual or hearing problems, headache, slurred speech
Can progress to seizures or coma
Managed - glucose tablets (Lucozade) or sugary drink or glucogel
If severe - call ambulance!
```
159
What monitoring should be undertaken for diabetic children?
```
Injection sites - lipohypertrophy
Annual bloods - T1DM associated with coeliac and thyroid disease
Eyes - retinopathy
Urine - nephropathy
Feet - neuropathy
Blood pressure
```
160
When is hypoglycaemia present in non-diabetics? What investigations would you do for hypoglycaemia?
Neonates- common in 1st few day of life
BLOOD GLUCOSE - defined by <2.6
Growth hormone, IGF-1, cortisol, insulin, C-peptide, fatty acids, ketones, glycerol, branched-chain amino acids, acylcarnitine profile, lactate, pyruvate
URINE after hypoglycaemia - organic acids
Save blood and urine for toxicology
161
What are the risks of prolonged hypoglycaemia?
neurological - epilepsy, learning difficulties, microcephaly - most likely in early childhood
Infants should never be starved for more than 4hr
DEFG!! - sepsis, unwell, prolonged seizure, altered state of consciousness
162
How is severe hypoglycaemia managed?
2ml/kg 10% dextrose, then 10% dextrose infusion
Avoid XS in case cerebral oedema
Delay in response - give IM glucagon
163
What are the different causes of congenital hypothyroidism?
Maldescent of thyroid and athyrosis - commonest - thyroid fails to develop or descent from base of tongue in early foetal life - lingual mass or unilobular small gland
Dyshormonogenesis - inborn error of thyroid hormone synthesis, commoner consanguineous
Iodine deficiency - commonest worldwide, iodise salt to prevent
TSH deficiency - associated with panhypopituitarism
164
What are the features of congenital hypothyroidism?
Often asymptomatic and picked up on screening, symptoms more prominent with age
Failure to thrive, feeding problems, delayed development
Prolonged jaundice
Constipation
Pale, cold, mottled, dry skin, coarse facies
Large tongue
Hoarse cry
Goitre (sometimes) - dyshormonogenesis especially
Umbilicial hernia
165
How is congenital hypothyroidism picked up on screening?
Day 5 newborn heel-prick test (Guthrie test)
Detects raised TSH
Defects due to pituitary abnormalities not picked up as will have low TSH
166
How is congenital hypothyroidism managed?
Early treatment to prevent learning difficulties
| Lifelong oral replacement thyroxine - titrate dose to growth, TSH and T4
167
What is the usual cause and features of juvenile hypothyroidism?
Caused by autoimmune thyroiditis
Increased risk in Down or Turner syndrome + other autoimmune disorders, females
short stature, growth failure, cold intolerance, dry skins, bradycardia, pale puffy eyes, loss of eyebrows, goitre, slow-relaxing reflexes, constipation, delayed puberty, obesity, SUFE, learning difficulties
168
What is the commonest cause of hyperthyroidism in children? What are its features?
Commonest = Grave's disease - thyroid stimulating immunoglobulins
Eye signs less common
Anxiety, increased appetite, sweating, diarrhoea, weight loss, rapid growth in height, tremor, tachycardia, warm, goitre, learning difficulties, psychosis
TSH low, T3 and 4 raised
169
How is hyperthyroidism treated?
First line - carbimazole, propylthiouracil for 2yrs
B blockers for symptomatic relief
Risk of neutropenia with antithyroid medications
Then 2nd course of surgery - subtotal thyroidectomy
Radioiodine treatment
Follow-up to check for subsequent hypothyroidism
170
What are features of inguinal hernias in children?
Commoner in boys and on right side
almost always INDIRECT - patent processus vaginalis
Require urgent assessment
Inguinoscrotal tenderness and swelling, separate to testis on palpation, can't "get above it"
Irreducible = emergency
Reducible = only emergency if red or tender
May be reducible if analgesia and gentle compression
Surgery - ligation and division of processus vaginalis to reduce hernia
171
What are the features of a hydrocoele?
Asymptomatic scrotal swellings, often bilateral, sometimes blue
can "get above it" on palpation
Not an emergency - tends to resolve on own
If persist beyond 1.5-2yrs old - surgery
172
What is cryptorchidism?
Undescended testis/es at birth, more common in pre-term infants
173
When do testes normally descend?
By 3 months of age
No intervention before 6 months to allow time
Some argue wait until 1 year old
174
How would you manage a retractile testis?
None needed, testis normally stays in scrotum with age
| Follow-up as may re-ascend
175
Why might an orchidopexy be done?
Fertility - for palpable or impalpable undescended testes - aim to refer for assessment and surgery at 6 months, spermatogenesis affected if not in scrotum at correct body temperature
Malignancy - increased risk testicular malignancy if undescended, but early orchidopexy can return risk to normal
Cosmetic, psychological - if testis absent, can use prosthesis instead
176
What does impalpable testis suggest? How would you investigate?
May be in inguinal canal, intra-abdominal or absent
Bilateral impalpable - urgent referral in case of congenital adrenal hyperplasia!!!
USS - verify internal pelvic organs, possibly see if absent or intra-abdominal testis
Hormonal - serum testosterone in response to IM HCG
Laparoscopy - investigation of choice for impalpable testis
177
What are the features of testicular torsion?
ACUTE severe testicular, groin or lower abdominal pain, may vomit, tender groin lump, testes may be pulled up or horizontal lie
Any age - common in adolescents
May have previous self-limiting episodes in Hx
Must be relieved within 6-12 hrs from onset of symptoms
Surgical exploration mandatory unless torsion can be excluded definitely
If is torsion - untwist and fixate contralateral testis
178
What is torsion of testicular appendage?
Torsion of hyatid of Morgagni - embryological remnant on upper pole of testis
Typically pre-pubertal boys due to expansion with gonadotrophins. Pain over 1-2 days increase
Blue dot sign
Surgical exploration and excision of appendage
179
How would you treat cerebral oedema secondary to DKA or DKA treatment?
IV mannitol (diuretic) or hypertonic saline
180
What is the pathophysiology of congenital adrenal hyperplasia and its life-threatening features?
autosomal recessive, 21-hydroxylase deficiency
Blocks pathways for cortisol and mineralocorticoid production - cortisol deficiency increases ACTH causing excessive androgen production
XS androgens - ambiguous genitalia if female (bilateral impalpable testes presentation), male less symptoms so present later
Low mineralocorticoid/aldosterone 80% = Low Na and High K+, salt-losing adrenal crisis
Low cortisol - shock, hypoglycaemic, acidosis. Also stimulates ACTH rise which causes hyperplasia and XS androgens
181
How would you manage congential adrenal hyperplasia?
Hydrocortisone lifelong and Fludrocortisone (if salt-losing)
Use U+E and urine to monitor sodium
Salt
Insulin+ dextrose, or salbutamol
Protect from cardiac arrhtymia - calcium gluconate
Monitor skeletal growth, genital surgery for females to have intercourse
182
How would you check for congenital adrenal hyperplasia in girls with ambiguous genitalia?
Clitoromegaly, darkening of labia
| Check blood pH, sodium, potassium
183
What is diagnostic of a salt-losing congenital adrenal hyperplasia? What other electrolyte features present?
17-alpha-hydroxyprogesterone raised in blood
| Electrolytes: Low sodium, High potassium, metabolic acidosis, hypoglycaemia
184
What is premature sexual development?
Development of secondary sexual characteristics before the age of 8 in females or 9 in males
Precocious puberty, or thelarche or pubarche
185
How might precocious puberty be classified?
Gonadotrophin-dependent (central) - premature activation of hypothalamic-pituitary-gonadal axis
Gonadotrophin-independent (XS sex steroids)
186
What might you consider in precocious puberty of a female?
Often normal or familial - check puberty ages in mother
Organic causes - if pubic hair before breast development, consider XS androgens (CAH or androgen-secreting tumour)
Or if rapid onset or neurological symptoms/signs - neurofibromatosis?
USS - ovaries and uterus (multicystic? enlarging? are normal)
187
What might you consider in precocious puberty of a male?
Usually organic, particularly intracranial tumours
Bilateral testicular enlargement - intracranial
Small testes - adrenal cause
Unilateral enlarged testis - gonadal tumour
Cranial MRI to find tumour
188
How would you manage precocious puberty?
Detect underlying pathology e.g. MRI
Skeletal maturation monitoring
Address psychological/behaviorual difficulties
If gonadotrophin-dependent - GnRH analogues
If gonadotrophin-independent - find source of excess steroids, inhibitors of oestrogen or androgen given
189
What is premature pubarche?
When pubic hair develops before 8 in females or 9 in males with no other signs of sexual development
Accentuated normal maturation of androgen production by adrenal gland (ADRENARCHE)
Asian and Afro-Caribbean more common
Self-limiting usually
USS ovaries and bone age to exclude central precocious puberty
Urinary steroid profile - exclude CAH, adrenal tumour
Increased risk of PCOS later in life
190
What is the definition of delayed puberty?
Absence of pubertal development by 14 in females or 15 in males
(more common in males, often familial or due to dieting, excessive physical training)
Often short children, long legs but final target height will be reached
191
How might non-pathological delayed puberty be managed?
Not usually required
If wanted - oral oxandrolone young males- androgenic anabolic steroid induces growth catch up
Older males - low dose IM testosterone
Females - oestradiol
192
What is the determining gene for sex differentiation?
SRY on Y chromosome - determines testis synthesis with production of testosterone, dihydrotestosterone
absence of SRY - ovaries and female genitalia
193
Briefly give the 4 different types of disorders of sexual differentiation
Excessive androgen causing virilisation in female - CAH
Inadequate androgen action
Gonadotrophin insufficiency
Ovotesticular disorder of sex development
194
What are the different causes of inadequate androgen action?
Inability to respond to androgens - partial or complete androgen insensitivity syndrome
Can't convert testosterone to dihydrotestosterone - 5 alpha reductase deficiency
Abnormalities in synthesis of androgens from cholesterol
195
What are may cause gonadotrophin insufficiency?
Syndromes e.g. Prader Willi
| Congenital hypopituitarism - small penis, cryptorchidism
196
What causes ovotesticular disorder of sex development)
AKA true hermaphroditism
XX and Y-containing cells in foetus lead to both testicular and ovarian tissue being present, complex external phenotype - rare
197
What should be done if there is a disorder of sexual development when assigning sex?
Sex must not be assigned if genitalia abnormal until detailed assessment and discussed with parents
Birth registration delayed until this completed
- karyotype
- adrenal and sex hormone levels
- USS internal structures and gonads
may need laparoscopic imaging and biopsy of internal structures
Possible to decide female as easiest surgery but is controversial
198
Give the features of hypospadias
Failure for urethral development proximal to distal to complete
- Ventral urethral meatus (glans, shaft, perineum)
- Hooded dorsal foreskin - not fused ventrally
- Chordee - ventral curvature of shaft
Glanular may be cosmetic concern but more proximal = functional problems - micturition, erectile deformity
Consider disorders of sexual differentiation or GU anomalies first
Surgery to correct
199
What are the common features of Kallman's syndrome?
Hypogonadotrophic hypogonadism: Delayed puberty or amenorrhoea, smaller testicles or undescended testes at birth
Pubic hair may be normal due to adrenal function OK
Other features: anosmia, hearing impairment, renal impairment, poor balance, scoliosis etc.
Caused by deficiency in GnRH
Due to gene mutations, often no family history
200
What gestational ages at delivery are used to classify level of prematurity?
Extremely pre-term = <28 wks
Very pre-term = 28-32 wks
Pre-term = 32-37wks`
201
What birthweights classify into categories of severity?
Very low birth weight = <1500g
Extremely low birth weight = <1000g
Incredibly low birth weight = <750g
202
What is the pathophysiology behind Respiratory Distress Syndrome in premature neonates?
28 wks is when start surfactant production
Type II pneumocytes lining the alveoli are not matured and do not produce sufficient surfactant
Therefore alveoli collapse, causing decreased surface area for gas exchange
AKA: Hyaline membrane disease - due to proteinaceous exudate seen on histology
Alveoli membranes are also thicker and less in number in pre-term infants
203
How does respiratory distress syndrome present?
At delivery or within 4h
Tachypnoea (>60breaths/min)
Laboured breathing, chest wall recession, nasal flaring
Expiratory grunting, cyanosis if severe
204
How would you manage RDS in pre-term infant?
Clear airway, raised ambient oxygen, CPAP via nasal cannulae or ventilation
Surfactant therapy + antibiotics in case of infection
High flow humidified O2 may be used to wean
Monitor O2 sats, HR, RR, temperature, glucose, blood pressure, weight
Temperature control - plastic bag, radiant warmer, humidifed incubator
Arterial, venous, umbilical lines for drugs, fluids, resuscitation
CXR
Minimal handling
205
What classifies as an early neonatal infection and how would you manage it?
Early = <48hr after birth
Give to cover Group B strep and Listeria
IV Benzypenicillin and IV Gentamicin every 12 hrs
Measure peak gentamicin level
206
What classifies as a late onset neonatal infection and what organisms tend to cause it? How would you manage?
Late = >48hrs after birth
Usually nosocomial - coagulase negative staph or fungal
Initial - Flucloxacillin + gentamicin
If coagulase -ve or enterococci - vancomycin
or broad-spectrum - meropenem
If meningitis suspected - bulging fontanelle, opisthonos - ampicillin + cefotaxime
207
Why is hypoglycaemia common in neonates?
Glycogen stores low, difficulty absorbing milk or no feeds at first. Possible increased demand from infection, respiratory effort
Hypothermia can also cause hypoglycaemia
Intracranial bleed may also drop blood sugar
208
What capillary blood glucose are you aiming for in a neonate? Why is hypoglycaemia managed?
Aim for 2.5
Give them a sugar bolus or sugar with IV infusion fluids if already given 3ml/kg
Need to prevent hypoglycaemia as important for brain
209
How would hypoglycaemia be managed in a term infant?
Encourage breastfeeding and offer bottle feed if hypoglycaemia
Would expect weight to drop in first week of life
210
How would you manage feeding in a pre-term infant?
Enteral feeding as soon as possible - 0.5ml/kg/hr and increment by 0.5
BREAST MILK ONLY
<28wks: Trophic feeds <1ml/kg/hr milk to encourage peristalsis to prepare for enteral feeds - stimulates gut hormones - commence asap after delivery 1-2hrly
TPN - IV feeding to prepare for enteral
Only feed neonate if they are well
211
What is the risk of increasing feeds too rapidly in a premature infant? How is it investigated and treated?
Necrotising enterocolitis - Bacterial invasion of ischaemic bowel wall - feed intolerance, vomiting (possibly bile stained), gastric distension, stool may contain fresh blood, shock
XR - distended bowel, intramural gas and portal tract, perforation by transillumination of gut
Treatment - stop oral feeds, broad-spectrum antibiotics, parenteral nutrition, circulatory and ventilatory support if needed
Surgery to remove bowel if perforated
212
How is nutrition monitored in premature neonates?
Anthropometry - weight, length, head circumference, MUAC at least twice weekly
Blood glucose, urine and stool output
U+Es
LFT and lipid profile
213
What is a common respiratory complication of ventilation or high flow oxygen in premature neonates?
Pneumothorax - ventilated air leaks into pleural cavity, demonstrated by transillumination with fibre optic light on chest - chest drain
Bronchopulmonary dysplasia - due to pressure, oxgen toxicity and infection
214
Give some long-term complications of being a premature infant?
Retinopathy of prematurity - oxygen toxicity - retinal vessel proliferation + detachment
Refraction errors+ squints
Sensorineural hearing loss, physical ear abnorms
Bronchopulmonary dysplasia
Chronic lung disease, increased risk chest infection, asthma
GOR, inguinal hernias
Cerebral palsy, learning difficulties
215
How would you identify and monitor potential complications of prematurity?
Ophthalmological assessment every wk
Hearing test
Ask about birth history in infections, lung disorders
Check for intra-ventricular bleed e.g. PVL or hydrocephalus - MRI, cranial ultrasound
216
What is apnoea and bradycardia desaturation in premature infants?
Common in very low birthweight infants until 32wks
Breathing may stop 20-30s or occur over closed glottis
Identify possible underlying cause, but often due to immaturity of centrla respiratory control
Treat - stimulant/caffeine, CPAP if frequent
217
Describe the possible aetiology, risk factors for preterm brain haemorrhage
Haemorrhage visible on cranial ultrasound. Often occur in germinal matrix above caudate nucleus
Most occur within 72hr life, more common after perinatal asphyxia, RDS or pneumothorax
If occurs in ventricles may cause hemiplegia or hydrocephalus (VP shunt may be needed and can lead to cerebral palsy)
218
Give the features of periventricular white matter brain injury in preterm neonates
Periventricular white matter - follows ischaemia or inflammation with or without haemorrhage.
Often not visible on cranial ultrasound initially. If resolves within week, low risk of CP but if visible cystic lesions on USS after 2-4 weeks = loss of white matter. May do MRI initially to find lesions
Bilateral multiple cysts = periventricular leukomalacia with high risk spastic diplegia and cognitive impairment
219
What is bronchopulmonary dysplasia/chronic lung disease? What would an XR show? What is its management?
Still on oxygen at post-menstrual age of 36 wks
XR - widespread opacity, sometimes cystic changes
Some need prolonged artificial ventilation
Most weaned onto CPAP + ambient oxygen
Corticosteroid therapy short courses to wean (but risk of CP if long)
High risk of infection e.g. RSV, pertussis - intensive care
220
What are the physiological causes of newborn jaundice?
foetal Hb lifespan shorter
High Hb concentration at birth causing release of Hb from RBC breakdown
Hepatic bilirubin metabolism less efficient for first few days
Only physiological if other causes excluded
221
What level of bilirubin causes clinical jaundice in neonates?
80 or above
222
What are the causes of jaundice with onset <24hr of age?
Haemolytic disorders - Rhesus disease (severe + hepatosplenomegaly), ABO incompatibility, G6PD deficiency, spherocytosis
Congenital infection - bilirubin is conjugated + other abnormal signs - growth restriction, hepatosplenomegaly, thrombocytopenic purpura
223
What are the different causes of jaundice from 2days to 2weeks of age in neonates?
Physiological
Breast milk jaundice
Dehydration
Infection
Possible haemolysis
Bruising or polycythaemia will worsen jaundice
Crigler-Najjar - glucuronyl transferase deficient/absent causing VERY high levels unconjugated bilirubin
224
What is breast milk jaundice?
Breast-fed infants are more likely to have jaundice which is prolonged
Unconjugated hyperbilirubinaemia
Multifactorial - possibly due to enterohepatic circulation of bilirubin
225
Why might infection cause jaundice in a neonate after 2 days?
Unconjugated bilirubinaemia - due to dehydration, haemolysis, reduced hepatic function, increase in enterohepatic circulation
Investigate cause and always check for UTI
226
How is the severity of jaundice assessed in a baby?
Check all babies within first 72hrs of life
Blanch the skin with finger
Jaundice starts on head/face then spreads downwards
If clinically jaundiced - transcutaneous bilirubin meter, try to confirm with blood sample (do more if darker skin)
Plot linear rise on chart until plateaus to decide treatment. Low threshold for pre-terms
Hypoxia, hypothermia lower threshold. Avoid drugs that displace albumin from bilirubin e.g. sulphonamides, diazepam
227
How is jaundice in neonates managed?
Phototherapy = blue-green 450nm light converts unconjugated bilirubin into water-soluble pigment excreted in urine. Risks - temp instability, nursing disruption, macular rash, bronze discoloration if jaundice conjugated
Exchange transfusion =if bilirubin rises to dangerous levels. Blood removed and replaced with donor blood (twice the infant's volume is exchanged)
IV Ig - if RhD or ABO, this can reduce need for exchange transfusion if phototherapy not working
228
What is Kernicterus?
encephalopathy due to deposition of unconjugated bilirubin in basal ganglia and brainstem nuclei
When unconjugated bilirubin exceeds albumin binding capacity
Lethargy, poor feeding, irritability, increased musucle tone, arched back (opisthonos), seizures, coma
Survive - may have choreoathetoid CP, learning difficulties and sensorineural deafness
229
What is persistent jaundice of neonates and what is important to rule out?
Jaundice in >2 week olds or 3 weeks if preterm
| Biliary atresia!!
230
What are most prolonged jaundices in neonates due to?
Unconjugated hyperbilirubinaemia due to - breast milk jaundice, infection (UTI), congenital hypothyroidism
231
What are the causes of prolonged jaundice with conjugated hyperbilirubinaemia? What are the signs of conjugated hyperbilirubinaemia?
Dark urine, pale stools - conjugated
Hepatomegaly and poor weight gain
Causes: neonatal hepatitis, biliary atresia
232
What may be offered to mothers with suspected pre-term labour?
2 steroid injections 24 hrs apart (to mature lungs to produce surfactant)
If 24-29 wks + in labour - MgSO4 to protect brain development
233
What may cause respiratory distress syndrome in TERM infants?
```
Transient tachypnoea of newborn
Meconium aspiration
Pneumonia
Pneumothorax
Milk aspiration
Persistent pulmonary hypertension of newborn
Diaphragmatic hernia
RARE - TOF, pulmonary hypoplasia, airway obstruction, pulmonary haemorrhage, congenital heart disease, intracranial trauma/encephalopathy, severe anaemia, metabolic acidosis
```
234
What is the commonest cause of RDS in term infants?
Transient tachypnoea of the newborn
- delay in resorption of lung fluid, more common after CS
CXR - fluid in horizontal fissure
Usually settles in 1st day of life, but may need additional ambient oxygen. Exclude other causes 1st
235
What is meconium aspiration and how does it occur?
Meconium passage more common in longer gestation babies, may be due to foetal hypoxia. Can inhale thick meconium at birth
May start gasping and aspirate meconium before delivery
Meconium = irritant = mechanical obstruction and chemical pneumonitis, possible infection
Over-inflated lungs, patches consolidation + collapse
Pneumothorax, pneumomediastinum
Artificial ventilation required usually
May develop persistent pulmonary HTN
236
What are the risk factors for pneumonia as a neonate?
Prolonged rupture of membranes
Chorioamnionitis
Low birth weight
- give broad spectrum antibiotics whilst awaiting infection screen results
237
What babies are at increased risk of milk aspiration?
Preterm - RDS, neurological damage
Bronchopulmonary dysplasia often have GORD which increases aspiration risk
Cleft palate
238
What is persistent pulmonary hypertension of the newborn?
Associated with RDS, birth asphyxia, meconium aspiration, sepsis, may be primary too
Right to left shunting in lungs, atria and ducts
Cyanosis soon after birth
CXR - pulmonary oligaemia
Urgent echo - to check for congenital heart disease
Mechanical ventilation, inhaled nitric oxide
Sildenafil
High-frequency oscillatory ventilation
239
What occurs with diaphragmatic hernia in RDS of term infant?
Presents as failure to resuscitate or RDS
Left-sided herniation abdo contents
Apex beats, heart sounds displaced to right, poor air entry left chest
Vigorous resuscitation may cause pneumothorax
XR chest + abdo - diagnosis
NG tube + suction to prevent distension of thoracic bowel, then repaired surgically once stable
Often have pulmonary hypoplasia - mortality high
240
What is biliary atresia and how does it present? How is it managed?
Progressive destruction or absence of extrahepatic biliary tree and intrahepatic biliary ducts
Chronic liver failure and death unless surgery
Normal birthweight but fail to thrive, mild jaundice
Pale stools, dark urine
Hepatomegaly, splenomegaly after
Ix: fasting abdo USS, radioisotope scan, liver biopsy
Laparotomy - operative cholangiography is diagnostic
Mx: Kasai - bypass fibrotic ducts, hepatoportoenterostomy
Success rate diminishes with age. Complications: cholangitis, fat malabsorption. If fails, liver tranplant
May get cirrhosis, portal hypertension anyway
241
What are choledochal cysts?
Cystic dilatations of extrahepatic biliary system
May present infancy with cholestasis
Older - abdo pain, palpable mass, jaundice, cholangitis
Dx: USS, radionuclide scan
Mx: Surgical excision + Roux-en-Y anastomosis to biliary duct
Complications: Cholangitis, malignancy
242
What are the different causes of hypoxic-icshaemic encephalopathy?
Before or during labour/delivery
- failed gas exchange across placenta
- interrupted umbilical blood flow
- inadequate placental perfusion (maternal BP high/low)
- Compromised foetus - anaemia, IUFGR
- Failed cardiorespiratory adaptation at birth
Post-natally - Kernicterus, inborn error of metabolism
243
What are the clinical manifestations of HIE?
Occur up to 48h after asphyxia
Mild - irritable, XS response to stimulus, staring, hyperventilation, poor feeding
Mod - abnormal tone, movement, cannot feed, seizures
Sev - no spontaneous movements, no response to pain, hypotonia alternating hypertonia, prolonged seizures - refractory, multi-organ failure
244
How is HIE managed?
Respiratory support
recorded amplitude-integrated EEG
Anticonvulsants for seizures
Fluid restriction - transient renal impairment
Treat hypotension - inotrope support
Monitor+treat hypoglycaemia, electrolytes especially hypocalcaemia
245
What is the prognosis of HIE?
Mild - complete recovery
Moderate - if feeding + neuro exam OK after 2 weeks then OK. If persists, unlikely to recover fully
Severe - 30-40% mortality, 80% neurodevleopmental disabilities
MRI 4-14 days showing abnormalities - high risk of later cerebral palsy
246
What are some soft tissue injuries that occur at birth?
Caput succedaneum - bruising/oedema presenting part beyond margins skull bones, resolves days
Cephalhaematoma - haematoma from bleeding below periosteum, confined to margins skull sutures
Chignon - oedema/bruising from Ventouse
Bruising - face if presenting or genitalia/buttocks in breech
Abrasions - scalp electrodes, scalpel incision CS
Forceps marks - transient
Subaponeurotic haemorrhage - diffuse boggy swelling of scalp, blood loss severe, hypovolaemic shock, coagulopathy
247
What nerve palsies may occur due to birth?
Brachial nerve palsy - breech or shoulder dystocia e.g. Erb palsy (C5+6) or elevated diaphragm. Most resolve completely - refer orthopaedics if not resolved 2-3 months
Facial nerve palsy - compression face against ischial spine. Unilateral facial weakness, open eye. Usually transient or may need methylcellulose drops
Cervical spine nerve palsies sometimes
248
What fractures may occur during birth?
Clavicle - shoulder dystocia - easily recovers
Humerus/femur - breech or shoulder dystocia. deformity, reduced movement and pain on movement. Heal quickly with immobilisation
249
What causes cleft lip and cleft palate?
Cleft lip - failure of fusion of frontonasal and maxillary processes
Cleft palate - failure of fusion of palatine processes and nasal septum
Mostly inherited polygenically or syndrome, detected on antenatal screening
250
How is cleft lip and cleft palate managed?
Cleft lip - first week of life for cosmetic surgery or delayed
Cleft palate - surgery several months of age. Can make feeding difficult
Follow up - check for otitis media, MDT approach - avoid adenoidectomy as may make feeding and speech worse
251
What is associated with oesophageal atresia?
Tracheo-oesophageal fistula
Associated with polyhydramnios during pregnancy
VACTERL - vertebral, anorectal, cardiac, tracheo-oesophageal, renal, radial limb anomalies
252
How do you check for oesophageal atresia?
Wide calibre feeding tube passed and checked on XR to see if reaches stomach
253
How does oesphageal atresia present if not diagnosed at birth?
Drooling and persistent salivation
Cough and choke when fed
May have cyanotic episodes
Possible aspiration of gastric contents or upper airway contents
254
How is oesophageal atresia managed?
Continuous suction applied to tube passed into oesophageal pouch to reduce aspiration of saliva and secretions
255
How does small bowel obstruction in neonates present?
Antenatal screening OR
persistent vomiting (bile-stained if below ampulla of Vater)
Initial meconium passed, but then rest is delayed
Proximal lesion - soon after birth, less distension
Distal lesion - later but more abdo distension
256
What can cause small bowel obstruction of the neonate?
- atresia or stenosis e.g. duodenal atresia in Down's
- malrotation with volvulus
- meconium ileus
- meconium plug
257
How are atresia/stenosis or malrotation of small bowel managed?
Correction of fluids and electrolytes
| SURGERY
258
How is meconium ileus managed?
Dislodged using Gastrografin contrast medium
259
How is a meconium plug managed?
Usually passes spontaneously
260
Briefly describe the causes of large bowel obstruction in neonates
Hirschsprung disease - absence nerve plexus in rectum which may extend along colon - does not pass meconium within 48h birth, abdominal distension. Acute enterocolitis may occur
Rectal atresia - absence of anus at normal site, If above levator ani muscle then fistula to bladder or urethra/vagina. Surgery required.
261
What is exomphalos?
Aka omphalocoele. Often antenatally diagnosed
Abdominal contents protrude through umbilical ring covered with transparent sac of amniotic membrane and peritoneum
Associated other congenital abnormalities
262
What is gastroschisis?
Bowel protrudes through defect in anterior abdo wall next to umbilicus - no covering sac. Not associated with other congenital abnormalities
High risk dehydration + low protein so wrap abdo in layers of clingfilm
NG tube and aspirated + IV infusion dextrose
Colloid support
Surgery: small = closure, large = silastic sac gradually returns
263
When might a TORCH screen be performed?
IUFGR
Suspected intrauterine infection/congenital infection
Intrauterine death
- can be taken antenatally from mother or postnatally from baby's blood
264
What does a TORCH screen test for?
```
Toxoplasmosis
Other - syphilis, VZV, parvovirus B19
Rubella
Cytomegalovirus
Herpes
```
265
How might you manage a baby with meconium aspiration on delivery?
Should not try to remove meconium while head on perineum - instead try to inflate lung within 1 minute
If floppy/apnoeic and thick meconiumm particulates, may be wise to check oropharynx and remove any obstructive bits
266
What is the first step in newborn resuscitation?
Team briefing and equipment check prior to birth
267
What occurs straight after baby is born in newborn resuscitation?
Dry baby, maintain temp, start timer
| Assess Tone, Breathing Rate, Heart rate
268
What should you do in newborn resuscitation if not breathing or is gasping initially?
Open airway, give 5 inflation breaths
SpO2 and ECG monitoring
REASSESS - heart rate and look for chest inflation
269
What should you do after 5 inflation breaths but reassess and chest not moving in newborn resus?
Re-check airway, head position
Repeat inflation breaths
SpO2 and ECG monitoring, look for response
270
What should you do in newborn resus once chest is moving?
Check heart rate - if undetectable or slow, ventilate for 30s
REASSESS
If HR still <60 - start chest compressions with ventilation breaths (3:1)
Reassess heart rate every 30 seconds
If remains low, consider venous access and drugs
271
How should chest compressions be done?
Hands round back of newborn with both thumbs meeting on lower third sternum - compress with thumbs
272
When are the two baby checks taken as part of the NIPE?
```
1st = within 72hr of birth
2nd = 6-8weeks
```
273
What 4 areas of the baby are examined in the NIPE?
Eyes
Heart
Hips
Testes
274
What are you looking for in the eye examination within NIPE?
Structural abnormalities, congenital cataracts - want to catch early as surgery is more effective early
Ophthalmoscope - red reflex absent/different between eyes = cataracts. If white reflex seen - retinoblastoma?
Eyes open, shape
Give safety netting for any unusual signs
275
What are risk factors for cataracts or visual impairments in newborns?
```
Maternal infection - rubella, toxoplasmosis, HSV
Family history childhood eye disorder
Low birth weight
Low gestational age
Herpes simplex virus
```
276
What classifies critical vs severe congenital heart disease?
```
critical = duct-dependent lesions requiring invasive intervension or leading to death in first 28 days
severe = requires invasive intervention or leads to death within 1 yr of life
```
277
What are risk factors for congenital heart disease?
Maternal infection e.g. rubella in 1st trimester
Family history congenital heart disease
Maternal conditions: SLE, T1DM
Medications during pregnancy - anti-epileptics, lithium
Down's, Noonan's, Marfan's syndromes
278
How would you refer in NIPE if find abnormal cardiac signs?
Newborn: urgent senior paed review
| 6-8wks: dicuss senior paed at time of exam regardless of symptoms
279
What are you checking for in the NIPE on the hips?
Developmental Dysplasia of the Hip
280
What are the risk factors for cryptorchidism?
Low birth weight or SGA
Low gestational age
1st degree relative with cryptorchidism
281
What should you do if find bilateral undescended testes in NIPE in newborn or infant?
Newborn - See senior paediatrician within 24 hrs, risk of endocrine disorder or CAH
Infant - check if already in pathway, if not, see senior paediatrician wtihin 2 weeks
282
What should you do if there is unilateral undescended testes at birth and at 6-8 weeks in NIPE?
Review at 6-8 weeks then if persists at 4-5 months
| Refer to surgeon if still not descended b 6 months
283
What syndromes are associated with hearing loss in children?
```
Usher's syndrome
Wardenberg's syndrome
Treacher Collins syndrome
Pendred syndrome
Alport syndrome
```
284
What congenital infections are associated with hearing loss?
CMV
Rubella
HSV
Syphilis
285
What groups are routinely tested for hearing and when?
All babies - UK Newborn Hearing Screening Programme (can miss mild losses) - Automatic Otoacoustic emissions within 4 months birth
All school children in reception
Hearing concerns, speech+language delay
CF, chemo, CMV, syndromes, head trauma, cleft lip+ palate, Downs syndrome
286
What tests are done to measure hearing?
Newborn screening - otoacoustic emissions
Auditory Brainstem Response done after if abnormal
Subjective tests depending on age:
6-18mnths - distraction test
6-30 mnths - visual reinforcement audiometry
30 mnths+ = performance testing or play audiometry
287
What is placenta-mediated disease?
Endovascular invasion failure - blastocyst invasion only reaches decidua and not maternal spiral arteries in myometrium. Spiral arteries remain low bore and high resistance leading to pre-eclampsia. Less blood flow to baby
288
What are the complications of placenta-mediated disease?
```
Pre-eclampsia
SGA or IUFGR
Placental abruption
Recurrent miscarriage
Premature birth
```
