Paediatrics 2 Flashcards
(13 cards)
Biliary Atresia
Congential condition - bile ducts narrow or absent.
Build-up of conjugated bilirubin = jaundice
Bile ducts transport bile liver > bowel
Bilirubin conjugated in liver, excreted in bile > urine/stool
Presentation of biliary atresia
-Physiological jaundice from day 2 - 7 due to increased breakdown and turnover of RBC = unconjugated bilirulin
- Biliary atresia = persistant jaundice > 14d in term babies and 21d in premature
- Biliary atresia = ↑conjugated bilirubin, physiolgical jaundice = ↑unconjugated bilirubin
Diagnosis and management of biliary atresia
1st line: blood test for conjugated and unconjugated bilirubin
1st line imaginig: USS
Mx:
- Kasai portoenterostomy = remove bile duct, attach small intestine to liver where bile duct was, bile in liver directly drains into intestines
- Liver transplant later
Hypoxic-ischaemic encephalopathy (HIE)
- Neonates, result of hypoxia during birth = ischaemic brain injury
- Results in cerebral palsy, developmental delays, cognitive deficits or death
Asphyxia (deprivation of O2) in brain = maternal shock, intrapartum haemorrhage, prolapsed cord
Diagnosis of HIE
- Hypoxia (during perinatal or intraparum period)
- Acidosis (pH < 7 or base deficit ≥ 12-16 mmol/L) on umbilical ABG
- Poor Apagar score (e.g. < 5 at 10min)
- Features of HIE
- Mild (grade 1) - resolve 24hrs
- Moderate (severe) - lethargy , hypotonia (flaccid), suppressed reflexes (absent), possible seizure, possible CP or developmental delay (90% possibility)
Management of HIE
- Neonatal ICU
- Supportive: neonatal resus and ongoing otipmal ventilation, circulatory support, nutrition, acid-base balance and tx of seizures
- Therapeutic hypothermia: 33 - 34c for 72hrs, then gradual warming over 6-12hrs
Cerebral Palsy
- Permenant neuro problems from brain damage at birth.
- Ranges from wheelbound and depedent on others for ADLs to subtle coordination/mobility problems
Causes:
- Antenatal: maternal infections, trauma
- Perinatal: birth asphyxia, prematurity
- Postnatal: meningitis, severe neonatal jaundice, head injury
Presentation of cerebral palsy
There are different types and patterns but I think it is too much detail
- Failure to meet milestones
- Increased or decreased tone - general or specific limbs
- Hanf preference < 18m
- Feeding/swallowing problems
- Learning difficulties
Popular OCSE station, CP signs are reliable and patients are stable. Get good at assessing and recongising patterns of UMN and LMN lesions
DDx of UMN lesions: brain injury or tumour
Management of cerebral palsy
- When asked “management will invovle a MDT approach”
- Physio - stretch and stregthen muscles
- OT - manage daily activites, adaptations, equipment
- SALT
- Dieticians
- Orthopaedic surgeons: release contratures and lengthen tendons (tenotomy)
- Paeds - muscle relaxant (baclofen), anti-epiletptic drugs, glycopyrronium bromide (drooling)
Conjuctivitis
- Purulent discharge, injection, eyelid swelling with first 48hrs of life = ?gonococcal infection
- Urgent gram-stain, culture, tx (e.g. IV 3rd gen cephalosporin) to prevent permenant vision loss
- Clamydia trachomatis = purulent discharge, eyelid swelling at 1 - 2 weeks old, tx: oral erythromycin 2w, tx mother + partner
Henoch-Schonlein Pupura (HSP)
IgA vasculitis - purpic rash lower limbs > buttocks, inflammation and blood leak from small blood vessels
Criteria (many, but EULAR/PRINTO/PRES criteria):
- Diffuse abdominal pain
- Arthritis or arthralgia
- IgA deposits on histology (biopsy)
- Proteinuria or haematuria
Clinical features of HSP
- Purpura (100%)
- Joint pain (75%) - knees, ankles
- Abdo pain (50%)
- Renal invovlement (50%) - IgA nephritis
Diagnosis and management of Henoch-Sconlein Purpura
Exclude serious pathology: meningococcaal septicaemia, leukaemia
- FBC and blood film: thrombocytopenia, sepsis and leukaemia
- Renal profile
- Serum albumin (nephrotic syndrome)
- CRP + Blood cultures (sepsis)
- Urine dipstick (proteinuria) + urine protein:creatinine ratio
- BP
Mx:
- Supprtovie with analgesia, rest + hydration
- Close monitoring in active disease: repeat urine dipstick (renal) and BP