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:

    • ophthalmoplegia

    • ataxia

    • areflexia

    • Ataxia predominantly affects the gait and trunk, with the limbs relatively spared

  • Acute motor axonal neuropathy (AMAN)

    • Attacks motor nodes of Ranvier

    • Prevalent in China and Mexico

    • Probably due to an auto-immune response directed against the axoplasm of peripheral nerves

  • 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

  • Preceding infections

    • 60% of cases do not have a known cause

    • Most common antecedent infection is Campylobacter jejuni

    • Followed by cytomegalovirus (CMV)

    • Some cases may be triggered by the influenza virus, or by an immune reaction to the influenza virus

  • Presentation

    • Ascending paralysis, weakness beginning in the feet and hands and migrating towards the trunk, is the most typical symptom

    • Some subtypes cause change in sensation or pain as well as dysfunction of the autonomic nervous system

  • Differentials

    • Chronic inflammatory demyelinating polyneuropathy (CIDP)

      • Acquired immune-mediated inflammatory disorder of the peripheral nervous system

      • Closely related to Guillain-Barré syndrome

        • Considered the chronic counterpart of that acute disease

    • Hereditary motor and sensory neuropathies (HMSN)

      • Group of neuropathies which are characterized by their impact upon both afferent and efferent neural communication

        • HMSN1 Charcot–Marie–Tooth disease type 1

        • HMSN2 Charcot–Marie–Tooth disease type 2

        • HMSN3 Dejerine–Sottas disease Charcot–Marie–Tooth disease type 3

        • HMSN4 Refsum disease 11213

        • HMSN5 Charcot–Marie–Tooth disease with pyramidal features / HMSN with pyramidal features — 600361

        • HMSN6 Charcot–Marie–Tooth disease type 6 / HMSN with optic atrophy 32095 601152

        • HMSN7 HMSN+retinitis pigmentosa

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

• • Affect Ras / MAPK signalling

  • Conditions:

  • Capillary malformation-AV malformation syndrome

  • Autoimmune lymphoproliferative syndrome

  • Costello syndrome

  • Hereditary Gingival fibromatosis type 1

  • Legius syndrome

  • LEOPARD syndrome

  • Cardiofaciocutaneous syndrome

  • Neurofibromatosis type 1

  • Noonan syndrome

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

  • https://docs.google.com/a/kieran101.com/open?id=1braurjMtMAgqDvZD6uGep9pyT-AtFDYOIpMv5jSCnXl_s4r-tzIprJ2N5Mh4

  • 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