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👉 *IMPORTANT 614* 👈 *A brief Introduction of Primary hyperaldosteronism* Primary hyperaldosteronism was previously thought to be most commonly caused by an adrenal adenoma, termed Conn's syndrome. However, recent studies have shown that bilateral idiopathic adrenal hyperplasia is the cause in up to 70% of cases. Differentiating between the two is important as this determines treatment. Adrenal carcinoma is an extremely rare cause of primary hyperaldosteronism. paceUrMRCP.

👉 *IMPORTANT 613* 👈 *Important points about Hypokalaemic periodic paralysis* *Symptoms* Limb weakness (unilateral) (40%) Arm weakness (unilateral) (40%) *Signs* Proximal muscle weakness (40%) *Investigations* Hypokalaemia (85%): The potassium is typically low during attacks   paceUrMRCP.

👉 *IMPORTANT 612* 👈 *Management of Addison,s disease* Patients who have Addison's disease are usually given both glucocorticoid and mineralocorticoid replacement therapy. This usually means that patients take a combination of: hydrocortisone: usually given in 2 or 3 divided doses. Patients typically require 20-30 mg per day, with the majority given in the morning dose fludrocortisone Patient education is important: emphasise the importance of not missing glucocorticoid doses consider MedicAlert bracelets and steroid cards discuss how to adjust the glucocorticoid dose during an intercurrent illness (see below) Management of intercurrent illness in simple terms the glucocorticoid dose should be doubled the Addison's Clinical Advisory Panel have produced guidelines detailing particular scenarios paceUrMRCP.

👉 *IMPORTANT 611* 👈 *Investigations of Addison's disease* In a patient with suspected Addison's disease the definite investigation is an ACTH stimulation test (short Synacthen test). Plasma cortisol is measured before and 30 minutes after giving Synacthen 250ug IM. Adrenal autoantibodies such as anti-21-hydroxylase may also be demonstrated. If an ACTH stimulation test is not readily available (e.g. in primary care) then sending a 9 am serum cortisol can be useful: > 500 nmol/l makes Addison's very unlikely < 100 nmol/l is definitely abnormal 100-500 nmol/l should prompt a ACTH stimulation test to be performed Associated electrolyte abnormalities are seen in around one-third of undiagnosed patients: hyperkalaemia hyponatraemia hypoglycaemia metabolic acidosis paceUrMRCP.

👉 *IMPORTANT 610* 👈 *Clinical features of Addison's disease* lethargy, weakness, anorexia, nausea & vomiting, weight loss, 'salt-craving' hyperpigmentation (especially palmar creases), vitiligo, loss of pubic hair in women, hypotension, hypoglycaemia hyponatraemia and hyperkalaemia may be seen crisis: collapse, shock, pyrexia paceUrMRCP. 👉 *IMPORTANT 610* 👈 *Clinical features of Addison's disease* lethargy, weakness, anorexia, nausea & vomiting, weight loss, 'salt-craving' hyperpigmentation (especially palmar creases), vitiligo, loss of pubic hair in women, hypotension, hypoglycaemia hyponatraemia and hyperkalaemia may be seen crisis: collapse, shock, pyrexia paceUrMRCP.

👉 *IMPORTANT 609* 👈 *Pathophysiology of Addison's disease* Autoimmune destruction of the adrenal glands is the commonest cause of primary hypoadrenalism in the UK, accounting for 80% of cases. This is termed Addison's disease and results in reduced cortisol and aldosterone being produced. *Other causes of hypoadrenalism* Primary causes tuberculosis metastases (e.g. bronchial carcinoma) meningococcal septicaemia (Waterhouse-Friderichsen syndrome) HIV antiphospholipid syndrome *Secondary causes* pituitary disorders (e.g. tumours, irradiation, infiltration) Exogenous glucocorticoid therapy paceUrMRCP.

👉 *IMPORTANT 609* 👈

👉 *IMPORTANT 608* 👈 *Few options for management of polycythemia vera* Phlebotomy – To keep hematocrit below 45% Aspirin – 81 mg daily Cytoreductive therapy – For patients at high risk for thrombosis Splenectomy in patients with painful splenomegaly or repeated episodes of splenic infarction Hydroxyurea is the most commonly used cytoreductive agent. If hydroxyurea is not effective or not tolerated, alternatives include the following: Interferon alfa Busulfan – In patients older than 65 years Ruxolitinib Fedratinib paceUrMRCP.

👉 *IMPORTANT 607* 👈 *WHO criteria for Polycythemia Vera* *Major* Hemoglobin > 16.5 g/dL in men and > 16 g/dL in women, or hematocrit > 49% in men and > 48% in women, or red cell mass > 25% above mean normal predicted value Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size) Presence of JAK2V617F or JAK2 exon 12 mutation. The *minor* WHO criterion is as follows: Serum erythropoietin level below the reference range for normal paceUrMRCP.

👉 *IMPORTANT 606* 👈 *Some possible findings in ploycythemia vera* Splenomegaly (75% of patients) Hepatomegaly (30%) Plethora Hypertension paceUrMRCP.

👉 *IMPORTANT 605* 👈 Impaired oxygen delivery due to sludging of blood in polycythemia may lead to the following symptoms: Headache Dizziness Vertigo Tinnitus Visual disturbances Angina pectoris Intermittent claudication paceUrMRCP.

👉 *IMPORTANT 604* 👈 The diagnosis of Motor Neuron Disease is clinically but we may do some tests to rule out other conditions... EMG will show acute denervation in MND. paceUrMRCP.

👉 *IMPORTANT 603* 👈 Some books suggest involvement of palliative care in Motor Neuron Disease from the time of diagnosis ( early involvement of palliative team ) paceUrMRCP.

👉 *IMPORTANT 602* 👈 Orthropeadic surgeon can be considered as a Jargon by some examiners so try to say joint surgeon. paceUrMRCP. 👉 *IMPORTANT 602* 👈 Orthropeadic surgeon can be considered as a Jargon by some examiners so try to say joint surgeon. paceUrMRCP.

👉 *IMPORTANT 601* 👈 Rheumatologist can be considered as a Jargon by some examiners so try to say joint doctor/specialist. paceUrMRCP.

👉 *IMPORTANT 600* 👈 Motor Neuron Disease usually does not affect sensations, autonomic system, eye muscles and vision paceUrMRCP.

👉 *IMPORTANT 599* 👈 To explain remissions and relapses of a disease say to the patient that this medical condition has a tendency to come in attacks and most of the time it comes and then gets better. paceUrMRCP.

👉 *IMPORTANT 598* 👈 When ever you find a new born baby in any scenario, ask about his well being you may get some good marks. paceUrMRCP.

👉 *IMPORTANT 597* 👈 For History taking make it a habit to say sorry to the patient when ever you hear presenting complaint from the patient. paceUrMRCP.

👉 *IMPORTANT 596* 👈 Gastroentrologist can be considered as a Jargon by some examiners so try to say bowel specialist. paceUrMRCP.