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👉 *IMPORTANT 619* 👈 *Brief management plan for Primary hyperaldosteronism* If adrenal adenoma: surgery If bilateral adrenocortical hyperplasia: aldosterone antagonist e.g. spironolactone paceUrMRCP.

👉 *IMPORTANT 618* 👈 *Few investigations for Primary hyperaldosteronism ( Conn,s Syndrome )* the 2016 Endocrine Society recommend that a plasma aldosterone/renin ratio is the first-line investigation in suspected primary hyperaldosteronism should show high aldosterone levels alongside low renin levels (negative feedback due to sodium retention from aldosterone) following this a high-resolution CT abdomen and adrenal vein sampling is used to differentiate between unilateral and bilateral sources of aldosterone excess Adrenal Venous Sampling (AVS) can be done to identify the gland secreting excess hormone in primary hyperaldosteronism paceUrMRCP.

👉 *IMPORTANT 617* 👈 *Few clinical features of Primary hyperaldosteronism ( Conn,s Syndrome )* hypertension hypokalaemia muscle weakness this is a classical feature in exams but studies suggest this is seen in only 10-40% of patients alkalosis paceUrMRCP.

👉 *IMPORTANT 616* 👈 *Few pearls for Vasovagal syncope explanation* Occurs when you faint because your body overreacts to certain triggers, such as the sight of blood or extreme emotional distress. It may also be called neurocardiogenic syncope. The vasovagal syncope trigger causes your heart rate and blood pressure to drop suddenly. That leads to reduced blood flow to your brain, causing you to briefly lose consciousness. Vasovagal syncope is usually harmless and requires no treatment. But it's possible that you may injure yourself during a vasovagal syncope episode paceUrMRCP.

👉 *IMPORTANT 615* 👈 *Few points about primary biliary cholangitis* is considered an autoimmune disease, which means your body's immune system is mistakenly attacking healthy cells.in this condition bile ducts which are slender tubes that carry the digestive fluid bile are destroyed. Bile ducts connect your liver to your gallbladder and to your small intestine. slender tubes (bile ducts) carry the digestive fluid bile. bile can back up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis). paceUrMRCP.

👉 *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.