Conditions Flashcards
Biliary atresia
Congenital narrowing of bile ducts . Jaundice >14 days term vs >21 days neonate. Kasai procedure. Increase in Conjugated bilirubin detected by direct bilirubin test.
Pyloric stenosis
Projectile vomiting and failure to feed and thrive in first few weeks of life. USS abdo and feel olive shaped mass. Hypochloraemic metabolic alkalosis. Ramstedt operation
Sandifers syndrome
Torticollis and dystonia after feed . Complication of GO reflux
Causes of bilious vomiting
Intestinal obstruction
Meconuim ileus
Hirchsprungs disease
Intussecption
Malrotation with volvulus
Imperforate anus
Oesophageal or duodenal atresia
Hirchsprung’s disease
Congenital absence of PNS ganglionic cells in myenteric/auerbach plexus in distal bowel and rectum. FMHX. >24 hrs delay in meconium. 2-4 weeks after birth 20% get hirchsprungs associated entero colitis- fever, abdo distension, sepsis, bloody diarrhoea —> toxic mega colon. RECTAL BIOPSY and abdo x ray
Intussception
6mnths-2yrs old. Associated with viral illness, HSP, polyps, meckel’s diverticulum. Sev colickly pain, red currant jelly stool, sausage shaped mass RUQ. USS. Therapeutic enemas/ surgery
Ileocecal valve is common place and most dangerous
Ileo ileo and cecocecal can sometimes self resolve
Lymphoma, appendix, are common lead points
Causes of short stature and investigations
Normal: familial, CDGP (delayed bone age)
Endo: GH deficiency, hypoT, cushings
Genetic: DS, turner’s, noonan, prader-Willi
Bone: achondroplasia, osteogenesis imperfecta, rickets
Chronic: coeliac, Ibd
IGF-1, predicted height, bone age, growth velocity
Causes of tall stature
Familial, obesity, GH excess, hyperT, precocious puberty, soto, Marian, homocystinuria, klinefelter, fragile X
Precocious puberty define and causes
Secondary sexual characteristics <8 yrs for girls or menarche <9yrs or <9yrs boys
Central: hydrocephalus, lesions
Peripheral: CAH, hypoT, gonadal or adrenal tumours (small testes), mccune Albright syndrome, testotoxicosis
Prematurethelarche
Delayed puberty
No breast dev by 13yrs. Primary amenorrhoea if no periods or secondary sexual characteristics at 14 vs if secondary sexual characteristics present then at 15. Boys testes <4ml at 14 yrs old.
Central: hypogonadotrophic hypogobadism - CDGP, chronic disease, hypoT, Kallman syn, prader-willi
Hypergonadotrohic hypogobadism- cryptochordism, androgen insensitivity syndrome, galactosasmia, thalassaemia,turners, klinefelters
Innocent murmur
Soft, systolic, symptomless, short, situational
Pansystolic murmurs
VSD ( left lower stern also border)
Tricuspid regurgitation
Mitral regurgitation
Ejection systolic murmurs
Aortic stenosis (right 2nd ICAS)
Pulmonary stenosis (left 2nd ICs)
HCOM 4th ICS left
Mid systolic murmur
ASD- crescendo decrescendo at UPPER LEFT STERNAL BORDER with a fixed split 2nd heart sound
PDA murmur
Continuous machinery crescendo decrescendo murmur
Diagnosis echo
Monitor until 1 yr and NSAIDs to close
If no closure after one year then surgery
Coarctation of aorta
Associated with TURNER’s SYNDROME.
3 days post birth collapsed baby when PDA closes. Weak femoral pulses, lower BP in legs, systolic murmur left infraclavicular and below left scapula. PG infusion
Pulmonary stenosis associations
Tetralogy of fallot
Rubella
Noonan syndrome
William syndrome
Tetrology of fallot
Pulmonary stenosis, VSD, RVH, overriding aorta
Cyanosis
Associated with rubella, alcohol, diabetes, increased maternal age
Echo BOOT SHAPED HEART
Ejection systolic murmur
Tet spells : squat, O2, B blockers, morphine, IV fluids, phenylephrine, NaHCO3
Pg infusion and aurgwry
Cyanosis heart disease
Truncus arteriosus
Transposition of great arteries
Triscuspid atresia
Tetrology of fallot
TAPVR
What heart disease is rubella associated with?
PDA
What syndrome is associated with AVSD?
Down’s syndrome
What heart disease is associated with turners syndrome?
Coarctation of aorta
Bronchiolitis
RSV under 1 yo, peaks at 6mnths
Coryzal symptoms that go to chest 1-2 days later.
Admit if under 3 months, pre existing condition, 50-75% normal intake, clinically dehydrated, RR>70, O2<92, signs of mod-severe respiratory distress, apnoeas
Management
NG / IV FLUIDS
SUCTION/ SALINE DROPS
O2- high flow- cpap - intubation
Viral induced wheeze
1-2 days fever/cough then goes to chest- resp distress and widespread expiratory wheeze.
Vs asthma- no atopy
Vs bronchiolitis- can occur up to 3yrs
RSV / rhinovirus
Same management as acute asthma- if <92% 3 BTB salbutamol and ipatropium nebs, O2 and steroid. If >92% salbutamol 10 puffs
Acute asthma
Mod: PEFR>50% predicted, normal speech
Sev: PEFR<50% predicted, can’t complete sentences, O2>92%, RR >40 if under 5yo vs >30 if >5yo, HR >140 if under 5yo vs >125 if over 5yo.
Life threatening: PEFR <33% predicted, <92% O2, silent chest, cyanosis,
Management for mod- sev
- 10 puffs/2 hrs salbutamol
- salbutamol and ipatroium back to back Nebs up to x3 if <92% (5mg/500mcg)
-pred/dex
-IV hydrocortisone
-IV MgSO4
-IV salbutamol
-IV aminophylline
Step down on 6 puffs / 4hrs 48 hours then 4/6hrs
Chronic asthma
2-3 yrs clinical diagnosis
-if improves on treatment
-spirometer with reversibility testing if >5yo , direct bronchial challenge with histamine, FENO, peak flow variability
Management
<5yo SABA, low dose ICS, leukotriene ant
5-12: salbutamol, low dose ICS, LABA, med dose ICS leukoyriene or oral theophylline, high dose ICS
> 12
SABA
low dose ICS
LABA
Medium dose ICS, leukotriene ant, oral theophylline, LAMA tiotropium
High dose ICS
oral steroids
Pneumonia
Productive cough & fever
Bronchial breath sounds coarse crackles
Strep pneumoniae
(Also staph aureus, h influenzae, mycoplasma, group a and B strep
RSV, influenza
Amoxicillin And macride
Croup
6mnths- 2 yrs
Parainfluenza
Not preceded by corzyal
Barking cough, strider, hoarse voice, low grade fever
Oral dex
O2
Nebuliser budenoside
Nebuliser adrenaline
Intubation
Strider causes
Foreign body
Croup
Epiglotitis
Laryngomalacia
Whooping cough
Bordetella pertussis
Mild cough/Coryza/fever —> severe paroxysmal cough (can cause vomiting or pneumothorax) with apnoeas and whooping
Notifiable disease
Macrolides 21 days or co trimoxazole
Complications: long cough or bronchiectasis
Chronic lung disease of prematurity
Risks :
<28 weeks
Required O2/ intubation at birth
RDS
O2 0.01L/min during sleep nasal cannula
Palizivumab injections
CF
AR CFTR Chr7 delta F508
1 in 2500
1 in 25 carriers
Doagnosis
Newborn blood test
Pilocarpine sweat test CL>60
CFTR
Mexonium ileus
Recurrent LRTI
steatorrhira
Chronic cough
Salty
Failure to thrive
Staph aureus
Pseudomonas and burkhoderia most dangerous
H influenza
E. coli
Klebsiella
Prophylactic flycloxacillun
Salbutamol
NEbulised DNAse
Nebuliser hypertonic saline
Creon
Monitor diabetes, osteoporosis, liver failure
HSP
under 10 yo
Purpuric rash on lower legs and buttocks
Abdo pain
Jt pain
Renal
UTI management
<3 months fever iv crftriaxone
>3mnthsnoral
Posterior urethral valve
Tissue proximal urethra near bladder causes bilateral hydronephrosis and UTIs
Usually picked up antenatal scan
Ablate
I descended testes
At 6-12 months orchidopexy
CMPA
under 3 years
Abdo pain and bloating and then allergy symptoms wheeze watery eyes cough rash
Perthes
5-8yo boys idiopathic
Disruption of blood flow to femoral head leads to avascular necrosis
Pain in groin/hip/knee, limp, restricted movement
X ray may be normal
Surgery if severe
5% get hip replacement
developmental dysplasia of the hip
Dislocation/subluxation of hip
RFs: breech, 1st degree FMHX, multiple pregnancy
USS diagnosis
Barlow and otolini test positive
Pavlik harness <6mnths vs surgery >6mnths w hip spica cast
SUFE
Adolescent obese male/ 8-15yo/ slight trauma causes head femur to slip on growth plate
Pain, external rotation , limp
Diagnosis X-ray frog lateral
Internal pinning and fixation of epiphysis