UaC

 =Unusual and Obscure Conditions and Treatments=



Week 3: Tocilizumab 
 -  A humanised anti-IL6 receptor monoclonal antibody o  Binds both soluble and membrane-expressed receptor  -  Interleukin-6 is a cytokine that is overexpressed in synovial tissue in patients with rheumatoid arthritis  *insert picture*

__Negative effects of IL-6__ - increased production of platelets - Increased chemokines and leukocytes - Increased synovial fibroblasts - Induce osteoclast activation - Damage to cartilage <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> __<span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Use at the WCH: __ <span style="font-size: 10pt; font-family: 'Arial','sans-serif'; mso-fareast-font-family: Arial;"> -  <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">SAS - compassionate use for systemic juvenile inflammatory arthritis <span style="font-size: 10pt; font-family: 'Arial','sans-serif'; mso-fareast-font-family: Arial;"> -  <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Made as an infusion in sterile <span style="font-size: 10pt; font-family: 'Arial','sans-serif'; mso-fareast-font-family: Arial;"> -  <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Patient has fortnightly 8mg/kg (360mg) administrations <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> __<span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Possible SEs: __ <span style="font-size: 10pt; font-family: 'Arial','sans-serif'; mso-fareast-font-family: Arial;"> -  <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Potentially immunosuppressant (can decrease neutrophils <span style="font-size: 10pt; font-family: 'Arial','sans-serif'; mso-fareast-font-family: Arial;"> -   <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">May increase transaminase and lipid levels <span style="font-size: 10pt; font-family: 'Arial','sans-serif'; mso-fareast-font-family: Arial;"> -   <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">GI disorders and skin rashes

Week 2: Letrozole
<span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- aromatase inhibitor used in hormone-receptor positive early breast cancer in post-menopausal women <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">androgens -aromatase---> oestrogens

__Negative effects of oestrogens__ --> maintain growth of hormone-dependent tumours <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> --> closure of epiphyses -> discontinued growth in  young men <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> __<span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Use at the WCH __ <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- letrozole can be used in adolescent males with short stature <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- inhibiting aromatase to reduce oestrogens and raise androgen levels delay the closure of the epiphyses, extending the period of growth <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> - usually in addition to testosterone <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- a study found after 18 months of treatment, the average predicted final adult height increased by 5.1cm <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- potential SEs: reduced BMD, metabolic effects including propensity for insulin resistance and dyslipidaemia

Week 1: Cysteamine
<span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> __<span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Used to treat cystinosis: __ <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- build up of cystine in the lysosome <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- due to inefficient cystine transport <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">-> crystallisation in kidneys, cornea, thyroid etc. <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- autosomal recessive <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> - affects 1 in 100 000-200 000 <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> __<span style="font-size: 10pt; font-family: 'Arial','sans-serif';">Mode of action: __ <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- cysteamine enters the lysosome <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- forms a complex with cystine <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- complex exits via lysine transporter <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">- once in cytoplasm, complex dissociates <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> - cysteamine can now re-enter lysosome <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">*can be used as eyedrops to treat corneal crystals <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> <span style="font-size: 10pt; font-family: 'Arial','sans-serif';"> <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">before treatment <span style="font-size: 10pt; font-family: 'Arial','sans-serif';">cystine build-up after treatment with cysteamine
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