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12.09.19 Ward round notes

Guillain-Barre Syndrome - 6 Types
  • Acute inflammatory demyelinating polyneuropathy (AIDP)
    • Most common form / often used synonymously
    • Caused by an auto-immune response directed against Schwann cell membranes
  • Miller Fisher syndrome (MFS)
    • Rare - 5% of GBS cases
    • Manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS
    • Usually affects the eye muscles first and presents with a classic triad:
    • Ataxia predominantly affects the gait and trunk, with the limbs relatively spared
  • Acute motor axonal neuropathy (AMAN)
  • Acute motor sensory axonal neuropathy (AMSAN)
    • Similar to AMAN but also affects sensory nerves with severe axonal damage
    • Like AMAN, it is probably due to an auto-immune response directed against the axoplasm of peripheral nerves
  • Acute panautonomic neuropathy
    • Most rare variant of GBS
    • Sometimes accompanied by encephalopathy
    • High mortality rate, owing to cardiovascular involvement, and associated dysrhythmias
  • Bickerstaff's brainstem encephalitis (BBE)
    • Characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski's sign
    • Large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla

Weird ECG stuff in kids
  • Heart rate >100 beats/min
  • Rightward QRS axis > +90°
  • T wave inversions in V1-3 (“juvenile T-wave pattern”)
    • Dominance of right ventricle
  • Dominant R wave in V1
  • RSR’ pattern in V1
  • Marked sinus arrhythmia
  • Short PR interval (< 120ms) and QRS duration (<80ms)
  • Slightly peaked P waves (< 3mm in height is normal if ≤ 6 months)
  • Slightly long QTc (≤ 490ms in infants ≤ 6 months)
  • Q waves in the inferior and left precordial leads.

Acute pancreatitis
  • Glasgow criteria: 3 of:
    • P02 Oxygen < 60mmHg or 7.9kPa
    • Age > 55
    • Neutrophilia White blood cells > 15
    • Calcium < 2 mmol/L
    • Renal Urea > 16 mmol/L
    • Enzymes Lactate dehydrogenase (LDH) > 600iu/L Aspartate transaminase (AST) > 200iu/L
    • Albumin < 32g/L
    • Sugar Glucose > 10 mmol/L


RASopathies


Pre- and Post- Ductal Sats

  • Difference of ≥10% suggests marked pulmonary hypertension or PDA dependent leison
  • Immediately after birth, post-ductal sats are lower due to unoxygenated blood coming across from the pulmonary artery
    • i.e. R => L shunt
    • Should normally disappear within 15 mins after birth
  • Pre-ductal > Post-ductal
    • Persistent pulmonary hypertension
    • Left heart problem
      • Aortic arch hypoplasia, critical aortic stenosis, interrupted aortic arch
    • NB These will tend to be more of a CYANOTIC picture but may be CARDIOVASCULARLY NORMAL
      • Unoxygenated blood comping across
      • But plenty of blood getting out to the body
  • Post-ductal > Pre-ductal
    • TGA
      • Small amount of oxygenated blood from a closed pulmonary loop in making it across to the aorta
    • CoA



Tanner stages


Notes
  • Atravent
    • Ipratropium bromide
    • Blocks the muscarinic acetylcholine receptors in the smooth muscles of the bronchi in the lungs, opening the bronchi
  • Key finding in tumor lysis syndrome
    • Hyperkalemia
    • Hyperphosphatemia
    • Hypocalcemia
    • Hyperuricemia / hyperuricosuria
    • Lactic acidosis
  • No evidence for salbutamol or ipratropium bromide in bronchiolitis
  • Wheeze in a young child?
    • Bronchiolitis UPO
    • See hyperinflation on CXR
  • Thymic shadow in infants' CXR
  • AVM
    • Lesion of the vasculature that develop such that blood flows directly from the arterial system to the venous system without passing through a capillary system
  • Oral baclofen and clonidine to treat children with spasticity
  • Kepra = Levetiracetam
    • Epilepay, esp. partial seizures
  • Acute asthma guidelines
  • Octreotide
    • Mimics natural somatostatin pharmacologically, though it is a more potent inhibitor of growth hormone, glucagon, and insulin than the natural hormone
  • Moro reflex
    • Present up to 4 or 5 months of age as a response to sudden falling
      • spreading out the arms (abduction)
      • unspreading the arms (adduction)
      • crying (usually)
  • ITP pharmacological management
    • Steroids
    • IVIG
    • Rituximab
    • NO aspirin / NSAIDs
  • MCV lower limit: 70 + age
  • Anaemia => Pica
  • Cyclic neutropenia
    • Tends to occur every three weeks and last three to six days at a time
    • Hereditary
    • Use G-CSF
    • esult of autosomal dominantly inherited mutations in ELA2, the gene encoding neutrophil elastase



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