Paeds Flashcards
APGAR SCORE
* carried out at 1, 5 and 10 mins post birth
* includes 5 aspects each with score 0-32
* what does a score of less than 3, 4-6, >7 indicate?
- Appearance: pink, blue peripherally, blue centrally
- Pulse: >100, <100, absent
- Grimmace (response to stimulation): good, little, none
- Activity (muscle tone): active, flexed limbs, floppy
- Resps: strong, slow, absent
Scoring
* <3: immediate resus
* 4-6: repeat every 5 mins
* 7: good
Signs of respiratory distress (facial, chest, sounds, obs)
Mx (maternal prevention & foetal mx)
Maintain prem neonates sats to …
-
Facial
1. nasal flaring
2. head bobbing
3. Cyanosis -
ChestRecessions
1. intercostal
2. supraclavicular
3. tracheal tug
Using accessory muscles of aspiration
Sounds: Abnormal airway noises
1. wheeze
2. stridor
3. grunting -
OBS x 3
1. Increased RR >60
2. Hypotension, systolic <70
3. Increased PR >160
Management
- Neonate: intubate or ventilate (endotracheal, biPAP, NIV) surfactant (LASA via tube) O2 (high-flow nasal cannula) +/- abx.
sats
91-95%
Necrotising enterocolitis
prem baby 1-2wks old; can progress to peritonitis –> sepsis, shock
* key features (4)
* Abdo XR findings (3) - related to gas - hallmark finding
* Mx (3)
- poor feeding, abdominal distension, bloody stools, vomiting
- gas in bowel wall; intramural (pneumostosis intestinalis); gas in peritoneum (pneumoperitoneum); gas in portal veins
- Sepsis 6; NBM; surgery (drainage/laparotomy)
Key features of retinopathy of prematurity
* Monitoring for babies <32wk
* treatment
- retinal vacularisation is stimulated by hypoxia and can take place up to 40 weeks.
- RoP - overoxygenation –> during further hypoxic event –> overcompensation and neovascularisation.
- Can lead to retinal detachment
- <32 week –> opthal review biweekly
- photocoagulation
Chronic lung disease of prematurity (aka bronchopulmonary dysplasia) presents with poor feeding &weight gain, IWOB, low O2 sats
- O/E
- seen most commonly in (2)
- Diagnosis can be made by ….
- prevention (maternal)
- req monthly injections with *Palivizumab *against which virus?
- crackles and wheeze on lung auscultation
- CXR or a baby requiring o2 past 36 weeks
- prem babies who require I&V
- Betamethasone if PTB lkely <36 weeks
- RSV
Neonatal abstinence syndrome
Treatment (weaned down) for
- opiate withdrawal
- non-opiate withdrawal
Which substance is most likely to present with symptoms weeks later (rather than within few days)?
- morphine
- phenobarbitone
- e.g. 21 days - Methadone
GBS sepsis
* 85% of cases present with (1)
* What is the preventative treatment and when is it administered?
* Which conditions require mother to take preventative treatment?
* Treatment to baby with confirmed GBS?
- Respiratory distress
- IV Benzylpenicillin to mother during labour
- mothers who contract GBS during current pregnancy or who previously had a baby infected with GBS
- Benzylpenicillin or Gentamicin
Distinguish early onset from late onset neonatal sepsis
Early - within 72 hrs
Late - after 72 hrs
Define transient hypoglycaemia of the newborn
Other causes for reduced glucose in newborns?
- maternal factors
- fetal factors
Management for prolonged/severe hypoglycaemia of the newborn
: if symptomatic, or glucose <1mmol (jittery, poor feeding, seizures, drowsiness..)
- if asymptomatic, low glucose but >1mmol
Blood glucose <2.6mmol without symptoms. Common and normal within first 24hrs of life.
Blood sugars drop due to the stress of birth; and after transition from maternal blood to their own blood.
Lowered glucose causes -
* maternal diabetes, beta blocker use
* fetal - large birth weight, BW syndrome
* increased demand - sepsis, HIE, hypothermia, prem.
Mx-
* 10% dextrose IV, NICU.
* Or encourage normal feeding (breast/bottle) and monitor blood glucose frequently (e.g every 2hours)
Neonatal jaundice
* Timings: normal vs abnormal
* Treatment (UCB vs CB)
* Prevents risk of ?
- Normal between 24 hrs and 2 weeks. Pathological <24s and prolonged >2 weeks.
- UCB -** photoisomerisation** - blue light therapy to convert UCB to isomers –> excretion via urine & bile.
- CB - surgery to rule out bilary atresia
- Kernicterus (high bilirubin levels in brain)
Neonatal jaundice treatment graphs
* If >50 below treatment line, no sx, no risk factors
* If <50: rules for at 18 hrs and 24 hrs
* If above first treatment line
* If above second treatment line, showing signs of acute toxicity or marked rise in bilirubin –>
- No need to repeat
- Repeat at 18 hrs (if RFs) or 24 hrs (if none)
- Phototherapy
- Consider exchange transfusion. NICU admission.
Distinguish the two antiglobulin tests (Coombs)
Direct (DAT) - haemolytic anaemia
Indirect (IAT) - prenatal testing & prior to blood transfusion
DDH
- Risk factor mnemonic
- Routine screening with Otolani & Barlow’s for all babies at newborn and 6-8 week check. Which babies require additional screening with ultrasounds (3)?
- Investigation options (2)
- Generally it stabilises within 6 weeks. What is the management for babies younger than 4-5mo, and if older?
Fing bollocks: First born, breech presentation, oligohydramnios, large birth weight, left hip, congenital calcaneovalgus foot deformity, >2.5kg, FHx, sex - male
* Breech >36 weeks
* FHx of DDH
* Multiple pregnancy
* Ultrasound or XR (>4.5m)
* Pavlik harness, or surgery (>4-5mo)
Congenital diaphragmatic hernia
* Abnormal sign O/E
* Most likely causes a Bochdalek hernia - what is this?
* Complications (3)
* Mortality rate
- tinking - bowel sounds auscultated within chest
- left sided hernia (posterolateral)
- pulmonary hypoplasia, HTN, RDS
- 50%
Distinguish oomphalocele from gastroschisis.
How does the mx differ?
Which is associated with Beckwith-Wiedemann syndrome and Down’s?
Oomphalocele is a defect of the umbilical cord whereby the gut contents herniate out of the body, into the peritoneal sac. Associated with BWS and Down’s. (or umbilical hernia).
- staged surgical repair over 6-12mo (reduce risk of resp compromise)
Gastroschisis is a defect of the abdominal wall where the contents herniate out without being covered by sac; exposed.
- cover with cling film (silo), return to theatre asap and surgically correct within 5 days
Pyloric stenosis
projectile vomiting 30 mins post feeding, visible persistalsis, results in
- which electrolyte abnormality?
- and post abo US what is surgical management?
- typical patient profile
- Metabolic alkalosis
- Ramstedt pyloromyotomy (good prog)
- White, caucasian males 2-4 weeks
CMPA/CMPI occur typically within first 3 months in formula-fed infants (or rarely breast fed infants later in life).
Distinguish cow’s milk protein allergy and cows milk protein intolerance.
Mx - maternal avoidance of dairy products. Breast feeding is encouraged; once breast-feeding stops what formula options are there (2), until 1yr, for at least 6 mo?
- Allergy - IgE mediated; normally resolves by 2-3
- Intolerance - non-IgE; 55% resolve by 5yrs.
- Both have symptoms of bloating, vomiting, diarrhoea
- but allergy also has symptoms like urticaria, wheeze and angio-edema.
- Options -** extensive hydrolysed formula (EHF - cow’s milk with porteins broken down; or AAF - amino acid formula)**
Meckel’s diverticulits
* rule of 2s
* gold standard imaging (if stable)
* presentation (2)
* treatment
- Meckel’s diverticulum is found in 2% of population, is found 2 feet from ileocecal valve, affects males:females 2;1, and is 2 inches long.
- rectal bleeding, abdominal pain similar to appendicitis
- Technetium-99-pertechnetate- a type of scintigraphy
- surgery if symptomatic
Meconium ileus
Presentation
Association
Abdo XRAY findings
Management (3 options)
Comparison to Hirschprung’s
Other differential
Delayed meconium >24hrs, abdominal distension and bilious vomiting
Cystic fibrosis
Dilated bowel loops, lack of fluid level, “soap bubble” or ground glass sign due to presence of air in the meconium
1. PR contrast study
2. NG NAC
3. Surgery
**Hirschprung;s **also presents with delayed meconium and abdominal distension and xray shows dilated bowel loops WITH FLUID LEVELS. Biopsy “gold standard” - aganglionic section of large bowel. Management is by rectal washouts or with anorectal pullthrough procedure.
Differential - meconium plug syndrome: preterm birth, use enema; diagnosis of exclusion
Breast-feeding
* encouraged by NICE until how many months - weaning?
* What is normal for the first 5 days but abnormal if carries on for 2 weeks?
* By what age should a child have doubled and then tripled in weight?
* Reduces risk of (2 examples for fetus, 3 for mother)
- 6
- to lose 10% of body weight. Still losing weight at 2 weeks ?FTT
- doubled by 5 months, tripled by 1 year
- Cardiovascular disease, diabetes
- Maternal - ovarian cancer, breast cancer, osteoporosis (reduced oestrogen plasma levels during breast-feed)
Assessing growth
* MPH & MPH target height (girls, boys).
* Method to record weight over time
* How to assess bone age?
- BOys = (maternal height + paternal height +14 / 2) Girls = maternal height + paternal height -14/2)
- <Target is MPH +/- 10cm
- Growth charts: age in mo, weight (x, y) -* if 25% centile, 75% of babies same age are heavier*
- Xray left wrist; assess bone age against chronological age - maturity obvious from epiphyseal centres
Short stature - genetic, acquired, FHX, constiutional delay.
Think pie chart for causes.
Genetic causes of short stature (4)
- Noonan’s
- Down’s
- Turner’s
- Prader-Willi
Developmental disorders
* HPG Axis
* Types of delayed puberty (2)
* and precocious (2)
*
- Hypo (GnRH;pulsatile) –> anterior pituitary (gonadotrophins - LH&FSH; pulsatile) –> gonads - testes and ovaries (sex hormones: testosterone, estradiol & progersterone).
Delayed
Hypergonadotrophic hypogonadism = excess FSH, LH due to impaired gonad function (inability to recognise gonatropins; no negative feedback to AP,)
Hypogonadotrophic hypogonadim = reduced FSH, LH due to impaired function of hypothal or AP.
Precocious - Central/true - HPG axis dependent - impaired HPG axis.
- Peripheral/pseudo - “ independent; excess sex hormones: e.g. congenital adrenal hyperplasia, gonadal hyperplasia, gonadotrophin release from intracranial lesion
Puberty
- Normal age range (boys, girls)
- First sign (boys, girls)
- Time to completed puberty (“)
- Delayed puberty
- Precocious puberty
Boys
* 10.5-13.
* Testicular enlargement.
* Delayed if >16.
* Precocious if <9
Girls
* 8.5-12.5.
* Thelarche.
* 2.5 yrs –> menarche –> ovulation (same time or mo later).
* Delayed if >13..
* Precocious if <8.
monitored TE with orchidometer.
DELAYED PUBERTY
Condition and levels of LH/FSH
* hypo
* hyper
gonadotropic hypogonadism
- CDGP
Hypo
- Low gonadotrophs = Low LH/low FSH
- Impairment in H or P of HPG axis.
- Congenital cause: **Kallmann syndrome: **
1. hypogonadotrophi
2. anosmia
3. X-linked
Hyper
* Klinefelters (XXY) and Turner’s (XO)
* Excess gonadotrophs = High LH/high FSH
* impairment in gonads to respond to gonadotrophs
* other causes = congenital adrenal hyperplasia (excess sex hormones produced from adrenals), gonadal tumour, gonadotrophin ectopic release
Turner’s syndrome (45XO) -
* Associations (cardiac, ENT, endocrine, intellectual)
* Typical appearance (5)
* Features
* Treatment (daily; and additional at 12-14 yrs)
- Coartation of the aorta, recurrent otitis media, hyperthyroid, obesity, intellectual disability
- Short stature, wide spaced nipples, webbed neck, cubitus valgus
- Delayed puberty; infertility in most (underdeveloped ovaries)
- GH daily + oestrogen/progesterone at puberty. Fertility treatment.
Examples of X-linked recessive conditions (A-W)
- remember no male to male transmission
- affected males - can have carrier daughters and unaffected males only
Examples of X-linked dominant conditions (3)
Androgen insensitivity syndrome (CAIS)
Becker’s and Duchenne’s muscular dystrophy
Colour blindness
G6PD deficiency
Haemophilia A and B
Nephrogenic Diabetes Inspidius
Retinitis pigmentosa
X linked dominant:
- Alport’s
- Rett
- VD resistant rickets
Growth hormone deficiency = pituitary dwarfism
* most frequent cause
* non-physical cause
* sign at infancy and in childhood
* daily SC treatment of:
- cranial radio therapy
- psychosocial deprivation - reversible after removal from adverse environment
- infancy - hypoglycaemia
- childhood - short stature & slow growth rate & delayed closure of anterior fontanelle
- rhGH = recombinant human growth hormone
Complete androgen insensitivity syndrome
* chromosomal makeup
* phenotype
* what surgery may be required due to increased risk of cancer?
* what is different about partial and mild types (PAIS and MAIS)
- 46 XY; X-linked condition
- male genotype but female phenotype; aka female genitalia but with testes rather than ovaries
- bilateral orchidectomy - testicular ca risk
- partial = ambigiuous genitalia
- mild = male genitalia but with female puberty traits e.g. breast development
Causes of tall stature
4 ps, 2ks, 2 ms
* pituitary adenoma
* parents
* puberty
* proteus syndromes
* Marfans
* MEN2
* Klinefleter
* Kallman
Opthalmia neonatorum - infection of newborn’s eye
* organisms
* mx
- chlaymidia/ neisseria gonorrhoea
- same day opthalmology/paeds assessment
Bronchiolitis
RFs: prem, immunosuppression, maternal DM, CHD.
* Presents with respiratory distress & signs of dehydration.
* Clinical course ?
* Criteria for considering admission (mainly supportive mx + high flow O2 if sats <92) (4)
* Criteria for immediate admission
* Common causative organism and preventative measure
* Monitoring required (2)
* Differential
- <3 mo
- Comorbidity
- Clinically dehydrated
- Feeding less than 50-75% normal intake.
Immediate
- severe respiratory distress (grunting, marked chest recession, RR >70)
- apnoea, central cyanosis, looks seriously unwell
- sats <20% persistently
Clinical course
* Coryza (1-2days) –> chest infection (3-7days) —> full resolution by 3 weeks.
* RSV
* Pavalizumab mAB.- monthly injection to high risk babies - not a true vaccine.
* Capillary blood gas (VBG) - monitoring Co2 and pH (respiratory failure).
* Viral induced wheeze - similar to Asthma but in children under 3 - episodic bronchoconstriction reversed by salbutamol.
Paediatric acute resp: condition and mx
* 1 yr old with barking cough, stridor, fever; caused by RSV/parainfluenza/adenovirus.
* 1.5yr old with 4Ds; in tripod positon; missed the 2 in 1 vaccine amongst others
* Few week old baby with noisy breathing and “ohm” sign on view through scope
* Child with paroxysmal coughing fits, inspiratory whoop, fainting, vomiting. Investigation options - early and late (2). Admit if 2 considerations. Abx for patient and for vuln contacts.
- Croup. Westley Croup Score for severity. Supportive (fluids+rest) or Dexamethasone/inh Budesonide
- Epiglottitis - HiB/diptheria. Drooling, dysphagia, dysphoria, distress. EMERGENCY - X examine/distress.* I+V; Cef & Dex.*
- Laryngomalacia - “floppy voice box”. Self resolves by 2.
- Pertussis - bordella pertussis. Nasal PCR or anti-pertussis toxin IgG test (if >2 weeks). ADmit if <6 mo, presence of severe sx (apnoea, cyanosis etc). Azithromycin.
Chronic paeds resp:
Cystic fibrosis: chronic autosomal recessive multi-organ condition due to mutation of CTFR gene; causing thickened secretions
* Investigations (3)
* First sign
* How does CF affect lungs, GI tract, endocrine, male reproductive organs
* MDT mx
* Differential - autosomal recessive condition associated with consanguinity.
- Newborn blood spot test, CTFR gene testing, sweat test (Cl>60)
- Meconium ileus (delayed meconium > 24hrs)
- Chronic cough; recurr. inf. Pancreatitis. Diabetes. steatorrheoa. Absence of vas deferens.
- SABAs and chest physio; CREON - enzyme replacment. Insulin therapy. Fertility treatment.
- General - high calorie diet, vaccinations (pneumococcal, influenza and varicella).
- Primary cilary dyskinesia - sinusitis, bronchiolectasias, reduced fertility. Kartagener’s syndrome is PCD with situs inversus.
more energy needed to breathe properly and fight off infections (immuno-compromised - require more calories)