Paeds Flashcards
Diagnosing T1DM in a child
Fasting glucose >/= 7
*Random blood glucose >/= 11.1
HbA1c >/= 48
Extra: Blood pH to exclude DKA Diabetes antibodies U&Es Autoimmune screen
1st presentation of T1DM1 management
Immediate
+
Long term
SAME DAY MDT paediatric diabetes team
Insluin ASAP
Admission for education + support
1) Insulin therapy: technique, dose adjustment
2) Nutritional: Complex carb high, <30% fat, 15-20% protein, refined sugar <25%
3) Monitoring of glucose: 4 x per day
Regualar management
- Avoid hypos
- Manage acute illness and avoid DKA
- Screen for micro and macrovascular complciations
Insulin need varies with age
Ongoing integrated package of care by MDT diabetes team
24 access to the team
Home-based care with support from local paediatric diabetes team
Initial inpatient management IF:
< 2 years
social/emotional factors
Home very far from hospital
Liase with school staff + education etc etc
Record details on Diabetes Register
Hypoglycaemia signs + cut off
Treatment
<4 blood glucose
Symptoms:
Hungry, dizzy, tummyache, faint, wobbly, progression to coma and seizures
Frequent episodes equals losing awareness of symptoms
Treating hypo
Early: sugary drinks or glucose tablet. Buccal if child unco-operative
Severe: glucagon injection kit if reduced consciousness (IM)
Unconscious: hospital to treat with IV glucose
After treatment biscuit/sandwich to prevent drop again
DKA management
Blood pH <7.3 + blood glucose >11.1, bicarb <15, capillary ketones >3mmol/l
A: Airway if coma. NGT if vom or coma
B: 100% oxgen face mask
C: Iv cannula, take bloods
- if shocked - 10ml/kg 0.9% saline bolus (Discuss more with consultant)
Diagnosis confirmation
Investigations: Blood glucose, Na, Cl, Ur, Cr, venous/cap blood gas
FLUIDS = requirement = deficit + maintenance
- Deficit 5% if ph>/= 7.1. 10% if <7.1.
- Maintenance if <10kg (2ml/kg/h), 10-40 (1ml/kg/h), more (fixed 40ml/hr)
FLUID = 0.9% NaCl (with 20 mmol/l KCl per 500ml)
Hourly rate = (10 x deficit/48) + maintenence/hour
BUT subtract boluses if >20ml/kg given before dividing
INSLUIN 1-2h after beginning IV fluid therapy
(dose 0.05-0.1 units/kg/hr)
Hourly monitoring: Cap glucose, fluid balance, GCS, vital signs
DKA risk
Cerebral oedema
Hypokalaemia
Aspiration pneumonia
T1DM follow up
4 x per year Regular dental Optician every 2 years Autoimmune screening Carry bracelet
Hypothyroid congenital vs acquired presentation
Congenital Usually asymptomatic and picked up on screening – faltering growth – feeding problems – prolonged jaundice – prolonged jaundice – constipation along – horse cry – delayed development – constipation along – hoarse cry – Congenital abnormalities e.g. heart defects
Acquired
SHORT STATURE
Classic
Females more than males
Why does neonatal hyperthyroidism occur
In infants of mothers with thyrotoxicosis from transplacental transfer of auntie TSH receptor antibodies – requires treatment as fatal but resolved with regression of maternal antibodies
CAH diagnosis
Presentation
Diagnosis
- 17-a-hydroxyprogesterone elevated for classical deficiency in 21 alpha hydroxylase
- 11-deoxycortisol if beta hydroxylase deficiency
DO blood gase \+ U&Es Genetic analysis Urinary steroids Pelvic and adrenal US for ambiguous genetalia at birth
Presentation
- F: At birth, virilisation
- M: salt losing crisis in the 80% that are salt loser ~ 1-3 weeks old = vom, weight loss, hypotonia, FTT
- -> Hypotension, hyponatraemia, hyperkalaemia, metabolic acidosis
Later features = Precocious puberty + final height below expected
Delayed puberty in female tests
Unlikely to be constitutional so do
- karyotyping
- TFTs
- Sex steroid hormones
- Consider eating disorder and pituitary pathology
Precocious puberty girl ultrasound
Uterus goes from tubular to pear shaped and can identify endometrial lining near menarche
Males causes of precocious puberty and how to differentiate
Differentiate based on testicular size
Bilateral enlargement (>l4m) = GDPP
Prepubertal testes = GIPP e.g. Adrenal tumour/CAH
Unilateral enlargement equals gonadal tumour
Normal puberty males
First sign is testicular growth at around 12 years of age (range = 10-15 years)
testicular volume > 4 ml indicates onset of puberty
maximum height spurt at 14
Normal puberty girls
first sign is breast development at around 11.5 years of age (range = 9-13 years)
Height spurt reaches its maximum early in puberty (at 12) , before menarche
Menarche at 13 (11-15)
Increase of only about 4% of height following menarche
Precocious puberty males
Secondary sexual characteristics <9
Precocious puberty causes (broadly)
- Gonadotrophin dependent (‘central’, ‘true’)
Due to premature activation of the hypothalamic-pituitary-gonadal axis
FSH & LH raised - Gonadotrophin independent (‘pseudo’, ‘false’)
due to excess sex hormones
FSH & LH low
Thelarche
Premature breast development
Typically between six months to 2 years
Rarely be on stage three puberty
Non-progressive and self-limiting
Adrenarche
Premature appearance of androgen dependent secondary sexual hair development acne and exhilarate odour
- Less than eight years in females
- Less than nine years in males
Causes of HIE
Biochem picture
[1] fail of gas exchange across the placenta - abruption, uterine rupture, excess contactions
[2] Interruption of umbilical bloodflow - prolapse, compressionl
[3] Inadequate placental profusion perfusion - PET, HTN
[4] Compromised fetus - IUGR, anaemia
Hyperoxia hypercarbia metabolic acidosis
Due to cardiorespiratory depression
Classification of H I E
How to diagnose
Mild: hyperventilation, hypertonia, Impaired feeding, responds excessively to stimulation
Moderate: movement abnormalities, hypotonic, cannot feed,? Seizures
Severe: no spontaneously response to pain, tone fluctuates, prolonged seizures, multi organ failure
Diagnosis needs
- Asses for intrapartum problen (e.g. CTG, prolapse)
– Resp depression at birth + need to resuscitate (Five minute Apgar <5)
– ACIDOSIS soon after birth (pH less than 7.0)
– Encephalopathy within 24 hours of birth
– Other causes of encephalopathy excluded
Management
- Resp suppor, AVOID HYPERTHERMIA (33-34 degrees best), fluid restrict, treat hypotension, monitor electrolytes and glucose, CFAM
Prognosis
- Mild - expect full recovery
- Moderate and feeding by 2 weeks = good prognosis
- Abnormalities persiting beyond 2 weeks = unlikely recovery
30% mortality if severe
What is PVL
Periventricular white matter lesions - echogencity, cysts = diagnosis on cranial US or MRI
- Due to severe HIE, poor cerebral perfusion, ischaemia, inflammation
Risk factors Extreme prematurity Hypertension Severely ill Hypercarbia
Prognosis
CP - SPASTIC DIPLEGIA LIKELY
Define with cause
Caput secuccedaneum
Cephalhaematoma
Chignon
Caput secuccedaneum
- Bruising and oedema of presenting part
- ACROSS SUTURE LINES
- above periosteum
- Resolves in a few days
Cephalhaematoma
- Haematoma from bleed below periosteum
- Condfined so doesn’t cross sutures
- Centre feels soft
- Resolves over several weeks
Chignon
- Oedema and brusing from Ventouse
Respiratory distress syndrome
Risk factors
Cause
Presentation
Management
- Oxygen sats to aim for and why
Risk factors
- PREMATURITY
- Male
- C-section
- 2nd of premature twins
- Diabetic mum?
Cause
- Surfactant deficiency –> alveolar collapse + inadequate gas exchange
Presentation
- WITHIN 4h
- Tachypnoea >60, Recession, nasal flaring, expiratory grunting, cyanosis if severe
Management - Oxygen (21-30%) --> 91% to 95% <91% = NEC + DEATH risk >95% = ROP
May need surfactant therapy or additional resp support
Prematurity pneumothorax causes
Symptoms
Diagnosis
Management
1) Premature (RDS)
2) Meconium aspiration
3) Pulmonary hypoplasia
4) Pneumonia
5) TTN
6) Mechanical ventilation
Clinical picture
- Reduced breath sounds and chest movement on affected side
Dx: Transillumination with fibre optic light
Management
- Chest drain if tension
- Otherwise resolves alone
- prevent with lowest pressure ventilaton
Causes of feeding difficulties/ weight loss >10% of newborn
Tongue… tell me more
Inexperienced mum
Tongue tie (ankyloglossia)
- M>F. Short lingual frenulum
- Symptoms - difficulty attaching/staying attached, unsettled, hungry all the time, clicking sound as they feed
- Management: nil if no feeding problems else tongue tie division (no anaesthetic if baby :O)
- Later if speech difficulties or feeding continues, same procedure but GA and stitches
Left subclavicular thrill Continuous murmur Bounding collapsing pulse Wide pulse pressure Heaving apex beat
Diagnosis
Risk factors
Management
Diagnosis
- PDA
Risk factors
- Congenital rubella
- Premature
- Born at high altitude
Echo to diagnose
Management if symptomatic
- Prostaglandin synthetase inhibitor e.g. INDOMETHACIn or IBUPROFEN to close duct
NEC clinical picture
cause
Characteristic findings
1st few weeks
Clinical picture – sudden change in feeding – vomiting (maybe be bile stained) – distended abdomen – store may have blood (fresh)
Cause
Bowel vulnerable to ischaemic injury and bacterial invasion in preterm infants
– can perforate –> Leaking into abdomen
Characteristic findings – Dilated bowel loops – bowel wall oedema and thumbprinting – Pneumastosis intestinalis (intramural gas) – If severe pneumoperitoneum
Management
STOP ORAL FEED AND GIVE BROAD ANTIBIOTICS
– Rest bowl
– energy to dream stomach, IV fluids and medicine
– surgery if perforation
Prognosis
Mortality of 20%
Newborn hypoglycaemia management
If 2 readings <2.6 inspite adequate feeds or 1 v low (<1.6) or symptoms
- IV glucose to maintain above 2.6
Jaundice risk factors
Tests to do
Sibling needing food therapy
Premature low-birth-weight
Exclusively breastfeeding
Jaundice in lesson 24 hours
Tests
<24h needs serum bilirubin in <2h
24h-2weeks needs serum bilirubin in <6h if TC reading >250
And other tests for less than 24 hours – blood group (mum and baby) – DAT – blood packed for volume – FBC, blood Film – G6PD levels – microbiology (blood you're in CFF if needed)
What is Kernicterus
Presentation
Long term sequelae
Deposition of unconj bilirubin in basal ganglia and brainstem
Presentation
- Unstable
- Opisthotonus
- Seizures
- Coma
Long term sequelae
- DYSKINETIC CP
- LD
- Sensorineural deafness
Biliary atresia
Progressive fibrosis and obliteration of extra and intra hepatic biliary tree –> leads to liver fibrosis failure and death within two years
Aetiology unknown
Mild jaundice, Pale stools and dark urine and faltering growth
Diagnosis Increase conjugated bilirubin is PT, INR (INR may be raised) Pepsi LFTs: GGT hight Ultrasound GOLD STANDARD = ERCP (lack of patency) WITH LIVER BIOPSY
Management
KASAI - same time as ERCP
= Hepatoportoenterostomy + Ursodeoxycholic acid
Low galactose 2 phosphate uridyl transferase on baby bloods
Diagnosis
Presentation
Treatment
Galactossoemia - can’t metabolise galactose –> identify in urine
Dx is low enzyme levels on blood
Features: Poor fed, vom, jaundice, heatomegaly
Treatment - no lactose
Alpha 1 antitrypsin deficiency
Diagnosis
Diagnosis
- <20 alpha -1 antitrypsin in plasma
Accumulation of protein in hepatocytes –> prolonged jaundice and bleeding due to vit K deficiency
Autosomal recessive
TTN
Delay in absorption of long fluid
More common after Caesarean
Chest x-ray make show fluid in horizontal Fissure
Usually settles within one day
When to suspect meconium aspiration
Management
Late born babies
Management only needed if respiration not established in 1 min
Complications
Airleak leading to pneumothorax and pneumomediastinum
Newborn pneumonia predisposing factors
Management
- prolonged rupture of membranes
- chorioamnionitis
- low-birth-weight
Management
Broad-spectrum antibiotics
Newborn pneumothorax predisposing factors
- meconium aspiration
- respiratory distress syndrome
- complication of mechanical ventilation
Persistent pulmonary hypertension of the newborn
Associations
Presentation
Birth asphyxia Meconium aspiration Septicaemia Respiratory distress syndrome Fluoxetine in 3rd trimester
Presentation
– Cyanosis soon after birth
Diagnosis: chest x-ray normal heart size
– urgent echo to exclude congenital heart disease and identify signs of raised pulmonary hypertension
Newborn coughing and choking when fed
Possible cyanotic episodes
Aspiration into lungs
Oesophageal atresia
- Associated with polyhdramnios
- Associated with oesophageal-tracheal fistula
- Absent stomach bubble on antenatal US
MUST SUCTION + surgery
What is port wine stain
Present from birth and usually grows with infant
- Vascular malformationin capillaries in dermis
- May be associated with Sturge Webber
- Disfiguring lesions can be treated with laser
What is strawberry naevi
Cavernous hemangioma
Usually not present at birth but appear in first month of life
More common in preterm infants
– increase in size until 3 to 15 months then regress
– ulceration or haemorrhage maker
– no treatment if small, topical propanolol may speed regression
DDH Risk factors
Describe the tests
Female Breach History First born Oligohydramnios
Barlow: Attempt to dislocate an articulated femoral head
- adduction of hip with posterior force on knee
Ortalani: Attempts to re-locate a dislocated family head
- Abduct hip with anterior force on femur
US ALL BREECH BABIES at 6 weeks
X-ray if >4.5months
Management
- Usually stabilise by 3-6 weeks spontaneously
- Pelvic harness if <4-5months
- Older children may require surgery
Croup
Presentation
Management
6m - 6yrs. Peak ~ 2yo
Viral - mostly parainfluenza
Starts with coryzal symptoms Stridor Barking cough Worse at night Hoarseness Variable difficulty breathing system include cyanosis, LOC, stridor, air entry,
Management depends on severity - scoring
1) Mild = no stridor at rest
- Oral single dose dexamethasone. Can go home
2) Moderate = stridor at rest; no agitation/lethary
- Oral single dose dexamethasone + Nebulised adrenaline
- Admit
3) Severe = stridor at rest; agitation/lethary
- Oral single dose dexamethasone + Nebulised adrenaline
- High flow O2
- Admit
Laryngomalacia
Define
Risk factors
Congenital abnormality of larynx cartilage – predisposes to supraglottic collapse during inspiration
– intermittent upper airway obstruction and strider
Risk factors – GORD – neurological – male – Genetics e.g. Down's syndrome
Usually resolves by 12 to 24 months
Management
– observe and treat GORD if mild
– endoscopic supraglottoplasty if severe
– tracheostomy is possible
Indication to admit a child with respiratory problems
Apnoea Saturation is less than 92% on air Fluid intake reduced (75% of usual) Severe Respiratory distress – Grunting – marked chest recession – respiratory rate greater than 70
Bronchiolitis
Presentation
causative organism
Dry wheezy cough Tachypnoea tachycardia Subcostal and intercostal recession Hyperinflation of the chest (liver displaced) Fine and inspiratory crackles High-pitched wheeze (expitatory mostly)
RSV
Whooping cough (pertusis)
Presentation
Risk factors
Complications
Diagnosis
Management
[1] A week of coryza (catarrhal phase)
[2] Characteristic paroxysmal or spasmodic cough followed by inspiratory whoop (paroxysmal phase). Lasts up to 3 months what the F – Worse at night – may vomit – go red or blue in the face – mucus from nose and mouth EPISTAXIS AND CONJUNCTIVAL HAEMORRHAGE
[3] SymptomsDecrease (convalescent phase)
Risk factors
– less than six months old
– lack of Vaccine
– contact
Complications – pneumonia – seizures – bronchiectasis Rare to get complications
Diagnosis
– I need a swab (identification of Bordetella pertussis)
– PCR: more sensitive
– FBC: Marked lymphocytosis over 15
Management
Admit and isolate if paroxysms of face
Antibiotics only work if started in catarrhal phase. ONLY GIVE IN 1st 21 days
<1 month old: seven days clarithromycin
>1 month old: azithromycin or clarithromycin
SCHOOL EXCLUSION
– Until 48 hours of antibiotics
– for 21 days after onset of symptoms if not treated
VACCINATE THE DAMN CHILD AFTER
+ PH ENGLANDfvs
Indications for tonsillectomy
Recurrent episodes of tonsillitis Peritonsillar abscess (Quincy) Obstructive sleep apnoea (Remove adenoids to)
Cystic fibrosis
pathology
Carrier rate: One in 25
Incidence: one and 2500 Live births
Autosomal recessive
Defect in the CF transmembrane conductance regulator (CFTR)
– CFTR = cAMP Dependent Chloride channel Found in cells that line the lungs, Intestine, pancreatic duct, sweat glands, reproductive organs
– CFTR gene is on chromosome 7
– Main mutation (78%) = deltaF508
Results in abnormal Ion transport
– Airway: reduced hourly service liquid layer, impaired celery function, retention of mucopurulent secretions
– Dysregulation of inflammation
– Interestine: Thick viscous meconium –> Meconium ileus in 10 to 20%
– Pancreatic duct: blocked my fix secretions –> Pancreatic enzyme deficiency and malabsorption
– Sweat glands –: XS sodium and chloride in sweat
Cystic fibrosis diagnosis
management
Newborn screen: IRT
– IRT high –> screened for common gene mutations
– If two mutations, sweat test To diagnose >60
Set test: low voltage current to pilocarpine applied to skin
– sweat collected
– Then confirm with gene abnormalities
NB: normal sweat test: Cl = 10-40
Also low faecal elastase
Faltering growth
Steatorrhoea
Management
ALWAYS MDT - specialist cystic fibrosis centre: physio, nurses, dieticians
– Physiotherapy at least twice a day to clear the Airway, regular nebulised hypertonic saline to decrease secretions
– Continuous prophylactic oral antibiotics (flucloxacillin) Plus additional rescue
– Pancreatic enzymes with all meals plus high calorie diet plus fat soluble vitamin supplements
weekly in their first month of life
- every 4 weeks when they are between 1 and 12 months old
- every 6 to 8 weeks when they are between 1 and 5 years old
- every 8 to 12 weeks when they are over 5 years
- old every 3 to 6 months as adults
Long-term picture with cystic fibrosis
Newborn
– screening
– meconium alias
Infancy – prolonged jaundice – growth filtering – recurrent chest infection – malabsorption, steatorrhoea
Young child – bronchiectasis
– rectal prolapse
– nasal polyps
– sinusitis
All the child/adolescent
– allergic bronchopulmonary aspergillosis
– DIABETES MELLITUS - check from age 10
– liver cirrhosis and portal hypertension
– distal intestinal obstruction (basically Maconi wireless)
– pneumothorax
– STERILITY IN MALES (absence of vas deferens)
Asthma management stepwise
[1] Diagnosis and assessment = SABA prn
[2] Using SABA 3 or more times a week
= + ICS v low dose
[3] Control still poor
5yo or more = + LABA
<5yo = + LTRA
[4] Control still poor in 5yo or more
Partial LABA response = + Increase ICS dose to low
No LABA response = STOP LABA + Increase ICS dose to low
[5] Control still poor. Consider
- Increase ICS dose to moderate
- Addition of theophylinne
REFER TO SPECIALIST
[6] Consider oral steroids
Hirschsprung’s disease
Cause
Where is the junction
Association
Presentation
Failure of ganglion cells to migrate into hindgut
= Absence of coordinated pal peristalsis an OBSTRUCTION is at the junction between normal bowel distal aganglionic bowel
80%: transition zone in rectum/sigmoid = short segement disease
20%: entire colon is involved = Long segment disease
Presentation – Usually first few days of life with lower intestinal obstruction 1. bile stained vomiting 2. .abdominal distension 3. failure to pass meconium
OCCASIONALLY CHILD PRESENTS with short segment disease and constipation
- Associated with trisomy 21
Investigation of Hirschsprung’s Disease
Management
Outcome
AXR: Obstruction but NOT SPECIFIC
CONTRAST ENEMA: Shows location of transition soon. Most valuable initial screening diagnostic test
Rectal biopsy: no ganglionic cells in submucosa
Initial management
– Bowel irrigation/rectal washout
Surgical treatment
– single stage pull through
Outcome
– 5% get normal bowel control
– 15 to 20% have partial control
– 5% never gain control (may have permanent stoma)
ENTEROCOLITIS = worst complication. 10% mortality
