Clinical 1 Flashcards
Prolonged PR on ECG is associated with:
Endocardial cushion defects (e.g. AVSD, ASD - or cardiac surgery in this area)
Ebstein’s
Acute rheumatic fever (carditis)
Congenital heart block (e.g. maternal lupus)
What are the biochemical abnormalities causing prolonged QRS?
Hyperkalemia
Hypothyroidism
Digoxin
What are the main medical things to think about with management of DMD?
- Medical treatment - STEROIDS
Surveillance and management of complications:
- mood and behaviour
- Cognitive impairment slightly more common - Wisckott score to identify areas requiring assistance and strengths, individualised learning plan for appropriate assistance with educational and physical needs
- CVS: cardiomyopathy - regular echo, treat if failure
- Resp: nocturnal failue - PSG + BiPAP. Don’t smoke, treat infections aggressively
- Ortho: Contractures/scoliosis - passive and active exercises, PT assisted programmes
OT for assisted supports, equipment, suitable home environment
Orthotist for orthotics, ortho for surgery
- Nutrition: diet/exercise
- Many will transition to adult services (get to 20s)
What are the social issues that are common in management of DMD?
Family support:
- end of life and palliative (life limiting illness) support from MDA for coping grief and loss
- Steroid side effects - behaviour - handling
- Avoid patronising and overprotective
- Workbridge - vocation planning
- Resources: Parent Project muscular dystrophy (int) or nzord.org.nz (NZ)
- Support with long-term goals (dating, work, etc)
What are the syndromes associated with dextrocardia?
Situs solitus or inversus (midline liver indicates situs ambiguous or heterotaxy)
Primary ciliary dyskinesia (Kartagener syndrome)
Heterotaxy: asplenia (Ivemarks) and polysplenia syndrome
What investigations do you want for dextrocardia and why?
ECG to review atria and ventricle orientation - needs to be reverse positions!
- normal lead placement leads to no R waves
- p axis and SA node in RA position
- q waves in V1/2 if L-TGA
- D-TGA associated with arrhythmia
Echo to review IVC and great vessels
AXR to determine situs
What are the corticosteroid side effects?
CUSHINGOID MAP
Cataract/cushingoid face – moon face/interscapular adiposity Ulcer Striae/skin thinning Hypertension Infection – thrush Necrosis of bone (avascular) Growth - short stature Osteoporosis/obesity Intracerebral pressure elevation (pseudotumour cerebri) Diabetes mellitus - glucose intolerance Myopathy(proximal)/moon face/mood Adipose tissue hypertrophy/acne Pancreatitis/psychosis – behavioural problems
What are the (non-cardiac) causes of hypertension?
MONSTER
Medications
Obesity (steroids/Cushing syndrome/phaeo)
Neonatal history
Symptoms / signs / syndromes (collagen, neurofibromatosis, tuberous sclerosis, SLE)
Trends in family (idiopathic)
Endocrine
Renal
White coat HTN
What causes isolated splenomegaly?
Remember “HIP”
Haematological
- Hereditary spherocytosis (autosomal dominant)
- G6PD deficiency (X-linked recessive)
Infective
- Typhoid
- Subacute bacterial endocarditis
Portal hypertension
What are the complications of splenomegaly?
Hypersplenism – thrombocytopenia
Functional asplenism – immunodeficiency and infection by encapsulated organisms
Splenic infarction
Early satiety (mass effect) causing poor growth
What is chorea caused by and what part of the brain is affected?
Occurs secondary to pathology affecting corpus striatum
Rapid movements: Occurs in: cerebral palsy, Sydenham’s Chorea, Wilson’s Disease, SLE, Moyamoya disease.
Also degenerative conditions such as: Ataxia telangiectasia, Huntington’s Chorea, Lesch Nyhan syndrome, PKU
What is athetosis caused by and what part of the brain is affected?
Athetosis (slow writhing movements, proximal extremeities)
Occurs secondary to pathology affecting the outer putamen
Can accompany chorea in dyskinetic CP, Wilson’s disease, Lesch-Nyhan, ataxia telangiectasia
What are the causes of dystonia?
Drugs (tardive dystonia), degenerative disorders such as Wilson’s, Hallervorden–Spatz diseases and hemiplegia
What are the causes of myoclonus?
Seizure disorder (infantile spasms, benign juvenile myoclonic epilepsy), degenerative conditions (neurocutaneous syndromes, Menkes, TaySachs, Wilson’s), structural brain anomalies (Aicardi syndrome, porencephaly), CVA, infections, and metabolic disorders
What is hemiballismus?
Unilateral random gross throwing movements of the proximal portion of the limb. Caused by contralateral subthalamic pathology
What are the causes of static tremor?
Wilson’s, Parkinson’s, Huntington’s, and Hallervorden-Spatz (pantothenate kinase disorder)
What are the causes of postural tremor?
Postural: most notable when arms outstretched in front of the body
Causes: thyrotoxicosis, phaeo, familial tremor, physiological tremor, Wilson’s
What are the causes of intention tremor?
Marked at the end points of movement. Causes include cerebellar pathology (and Wilson’s disease)
Hemiballismus
What are the causes of unilateral facial palsy?
- Pontine lesion - tumour/CVA/infection
- Posterior fossa - tumour/meningitis
- Peripheral nerve – GBS, Bell’s palsy, Ramsay Hunt, parotid gland tumour, petrous temporal fracture
- Ramsay Hunt – check ears for vesicles
What are the causes of bilateral facial palsy?
Moebius (absent CN VII +/- CN VI, can be unilateral, associated with Poland syndrome, intellectual disability, hypogonads)
Cerebral palsy
Neuromuscular junction (Myasthenia gravis, infant botulism (ptosis + opthalmoplegia)
Dystrophy - myotonic dystrophy (ptosis, distal weakness, myotonia > 5 years, myopathic facies, cataracts), facial-scapular-humeral dystrophy