pace your MRCP-PACES

*Announcement for Online Session No 63* *14 August 2022* DEAR DOCTORS : MAY I HAVE YOUR ATTENTION PLEASE : *Tomorrow we will have an online session on Zoom discussing 1 station 2 ( History Taking )* regarding our preparation for MRCP PACES ( UK ) TIMINGS : Saudia Arabia: 4 pm Pakistan : 6 pm Bangladesh : 7 pm India : 6 30 pm Singapore : 9 pm Hong Kong : 9 pm Malaysia : 9 pm Egypt : 3 pm Libya : 3 pm Bahrain : 4 pm Burma ( Myanmar ) :7 30 pm Sudan : 3 pm UAE : 5 pm UK : 2 00 pm Ireland ( Dublin ) : 2 00 pm Afghanistan : 5 30 pm Kenya : 4 00 pm Germany ( Berlin ) : 3 00 pm Nigeria : 2 00 pm Japan ( Tokyo ) : 10 00 pm Denmark : 3 00 pm Qatar : 4 00 pm Oman : 5 00 pm Italy : 3 00 pm ( please Google for your local time zones to avoid any inconvenience ) Zoom meeting link will be shared 5 minutes before start time. Interested candidate may send a personal message to take the case. GOOD LUCK.

*Few investigations for Wilson’s disease by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* Reduced serum caeruloplasmin Reduced serum copper (counter-intuitive, but 95% of plasma copper is carried by ceruloplasmin) increased 24hr urinary copper excretion Good Luck

*Some clinical features of Wilson’s disease by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* The onset of symptoms is usually between 10 - 25 years. Children usually present with liver disease whereas the first sign of disease in young adults is often neurological disease Features result from excessive copper deposition in the tissues, especially the brain, liver and cornea: liver: hepatitis, cirrhosis neurological: basal ganglia degeneration, speech, behavioural and psychiatric problems are often the first manifestations. Also: asterixis, chorea, parkinsonism, dementia Kayser-Fleischer rings renal tubular acidosis (esp. Fanconi syndrome) haemolysis blue nails Good Luck

*Some info about Wilson’s disease by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* Wilson's disease is an autosomal recessive disorder characterised by excessive copper deposition in the tissues. *Pathophysiology* Metabolic abnormalities include increased copper absorption from the small intestine and decreased hepatic copper excretion. Wilson's disease is caused by a defect in the ATP7B gene located on chromosome 13. Good Luck

*Few differentials for Jaundice by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* Gilbert's syndrome Biliary colic Ascending cholangitis Infectious mononucleosis Intrahepatic cholestasis of pregnancy Pancreatic cancer Hepatocellular carcinoma Primary biliary cholangitis Primary sclerosing cholangitis Hepatitis A Acute liver failure Cholangiocarcinoma Hepatitis B Autoimmune hepatitis Acute fatty liver of pregnancy Malaria (Falciparum) Hereditary spherocytosis Good Luck

*Some treatment options for Achlasia by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* pneumatic (balloon) dilation is increasingly the preferred first-line option,less invasive and quicker recovery time than surgery patients should be a low surgical risk as surgery may be required if complications occur surgical intervention with a Heller cardiomyotomy should be considered if recurrent or persistent symptoms intra-sphincteric injection of botulinum toxin is sometimes used in patients who are a high surgical risk drug therapy (e.g. nitrates, calcium channel blockers) has a role but is limited by side-effects Good Luck

*Important Investigations for Achlasia by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* oesophageal manometry excessive LOS tone which doesn't relax on swallowing considered the most important diagnostic test barium swallow shows grossly expanded oesophagus, fluid level 'bird's beak' appearance chest x-ray wide mediastinum fluid level Good Luck

*Some clinical features of Achlasia by courtesy of Dr Toqeer Bhatti. Thanks a lot dr :* dysphagia of BOTH liquids and solids typically variation in severity of symptoms heartburn regurgitation of food - may lead to cough, aspiration pneumonia etc malignant change in small number of patients Good Luck

*Some info about Achlasia by courtesy of Dr Toqeer Bhatti. Thanks a lot dr :* Failure of oesophageal peristalsis and of relaxation of lower oesophageal sphincter (LOS) due to degenerative loss of ganglia from Auerbach's plexus i.e. LOS contracted, oesophagus above dilated. Achalasia typically presents in middle-age and is equally common in men and women. Good Luck

*Few Differntials for ODYNOPHAGIA by courtesy of Dr Toqeer Bhatti. Thanks a lot dr*: Viral upper respiratory tract infections Acute tonsillitis Peritonsillar abscess (quinsy) Infectious mononucleosis Influenza Oesophageal cancer Acute epiglottitis Good Luck

*Few differential diagnosis of dysphagia by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* Laryngopharyngeal reflux Oesophageal cancer Eosinophilic oesophagitis Peritonsillar abscess (quinsy) Gastric cancer Achalasia Subacute (De Quervain's) thyroiditis Pharyngeal pouch Thyroid cancer Acute epiglottitis Ludwig's angina Myasthenia gravis Motor neuron disease Dermatomyositis Polymyositis Good Luck

*Few important complications of extrensic allergic alveolitis by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* Fibrotic and emphysematous changes following inflammation in the lung during an acute period may lead to fibrosis, which has long term effects. Studies have found that 20% of patients with farmer’s lung (EEA secondary to occupational exposure to hay dust or mould spores) developed emphysema. Chronic oxygen requirement Widespread fibrosis may result in chronic hypoxaemia requiring long-term oxygen therapy at home Decreased lung function will lead to a reduced ability to carry out physical activities and activities of daily living Good Luck

*Some info about management of extrensic allergic alveolitis by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* British Thoracic Society (BTS) guidelines on interstitial lung disease (2008) underline the key factor in management is the identification and avoidance of the precipitating agent. Identification by a thorough clinical history including occupational hazards and correlation with symptom timeline of the patient. A causative agent may not be able to be identified in up to 25% of cases BAL cytology or antigen testing of the blood may confirm the causative agent Once identified, the removal of the causative agent is the most important part of management, and symptoms should resolve following this. Liaison with the employer and occupational health if the agent is work-related, and with the provision of respiratory masks and other protective equipment may be necessary Corticosteroids A trial of corticosteroids e.g. oral prednisolone can help with symptoms, mostly in patients with equivocal clinical presentation, as EEA tends to be steroid-responsive, whereas idiopathic pulmonary fibrosis does not This should then be tapered over a period of several weeks Patients with chronic EEA may benefit from long-term low dose prednisolone Good Luck

*Important cliniical features of extrensic allergic alveolitis by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* *Clinical features* A thorough clinical history is essential. This must include an occupational history, as certain jobs are associated with exposure to agents that can cause EEA: Farmers Particularly mushroom and potato workers, compost workers Animal cleaning/breeding Particularly avian species Chemical industry Working with paints, powders Smelters and hard metal workers A particular focus on exposure at home or at work to moulds is important, including in ventilators, air conditioners, hay or wood as this is a common causative agent. The presentation may be acute, with symptoms presenting 4-8 hours after exposure to causative agent and subsiding within 24-48 hours, or chronic, usually following long term low-exposure to a causative agent. Acute EEA typically presents, alongside a suggestive occupational/exposure history, with: Cough (productive or non-productive) Dyspnoea Fever Malaise Chest tightness Acute type 1 respiratory failure may develop in severe cases Chronic EEA presents more insidiously, alongside a suggestive occupational/exposure history, with: Insidious cough/dyspnoea symptoms Weight loss Clubbing (50% of cases) More widespread fibrotic changes mimicking idiopathic pulmonary fibrosis On auscultation of the lungs in both cases, bilateral midzone inspiratory crepitations will likely be heard to a greater or lesser extent depending on the severity of the condition. Good Luck.

*Pathophysiology of extrensic allergic alveolitis by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* *Pathophysiology* It is thought to be largely caused by immune-complex mediated tissue damage (type III hypersensitivity) although delayed hypersensitivity (type IV) is also thought to play a role in EAA, especially in the chronic phase. Good Luck

*Some info about extrensic allergic alveolitis by courtesy of Dr Toqeer Bhatti. Thanks a lot Dr* Extrinsic allergic alveolitis (EAA, also known as hypersensitivity pneumonitis) is a condition caused by hypersensitivity induced lung damage due to a variety of inhaled organic particles. *Classification* Examples bird fanciers' lung: avian proteins farmers lung: spores of Saccharopolyspora rectivirgula (formerly Micropolyspora faeni) malt workers' lung: Aspergillus clavatus mushroom workers' lung: thermophilic actinomycetes Good luck

✌️✌️ *HEARTIEST CONGRATULATIONS* ✌️✌️ To *Dr. Tahseen Azim* For passing *PACES MRCP ( UK )* from UK. She was with us in our batches of Feb & July 22 online course for PACES. We wish her the best for her future.

*☝️ IMPORTANT 26 ☝️* *For clinical stations :* Please remember : When you *suspect pulmonary embolism* then you have to calculate *Modified Two level PE Wells Score* ( or atleast mention to the examiner that you will like to calculate it ) If score is *more then 4* go for immediate CTPA ( Or treat empirically with LMWH If there is delay in CTPA ) If score is *less than 4,* do D Dimers , if D Dimers come out to be positive go for immediate CTPA or empirical treatment with LMWH. If D Dimers are negative then consider alternative diagnosis.  Good luck.

*Few words about prognosis of Hodgkin Lymphoma by courtesy of Dr Toqeer Bhatti. Thanks a lot dr* The Ann Arbor staging system helps determine prognosis and the intensity of treatment, with stage I and II patients having a better prognosis. Stage I and II Hodgkin's lymphoma Certain prognostic factors subdivide stage I and II lymphoma into favourable and unfavourable groups, including the presence of B symptoms, extra-nodal involvement and high erythrocyte sedimentation rate (ESR) levels. Patients in stage I/II favourable group have a prognosis of around 85-90%, and that in the unfavourable group 80-90%, after receiving a combination of chemo- and radiotherapy treatments. Stage III and IV Hodgkin's lymphoma The prognosis of stage III/IV lymphoma is affected by a set of risk factors that correlate with poorer prognoses: male, age >45, leukocytosis, lymphopenia, low haemoglobin, low albumin and high ESR levels. The 5-year survival rate of patients in this group ranges from 42% (with >5 risk factors) to 84% (without any risk factors). Good Luck

*Few Complications of Hodgkin Lymphoma by courtesy of Dr Toqeer Bhatti. Thanks a lot dr* Complications of Hodgkin's lymphoma are usually chemotherapy or radiotherapy related. Thyroid disorders Around 50% of the patients who received radiotherapy experience symptoms of hypothyroidism. Other possible thyroid disorders include thyroid cancer and hyperthyroidism. Therefore safe netting and regular monitoring of thyroid function should be done in patients receiving radiotherapy. Chemotherapy drugs, especially alkylating agents are associated with secondary malignancies such as acute myeloid leukaemia and paraneoplastic syndrome. Secondary leukaemia is seen in around 3% of patients receiving the BEACOPP regimen (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone). Radiotherapy, on the other hand, is associated with an increased risk of solid tumours especially lung cancer and breast cancer. Cardiac abnormalities Patients receiving anthracyclines e.g. doxorubicin are at a higher risk of developing cardiomyopathy. Patients can develop acute pericarditis shortly after receiving radiotherapy, or valvular heart disease or coronary heart disease in the long-term (around 5-10 years post-radiotherapy). According to BMJ Best Practice (Oct 2019), baseline cardiac function should be established by performing echocardiogram or multi-gated acquisition (MUGA) scans before starting anthracyclines. The article also suggested administrating lower doses of radiotherapy as well as managing cardiovascular risk factors to minimise complications. Pulmonary toxicity About 20% of patients receiving the ABVD regimen (doxorubicin, bleomycin, vinblastine, dacarbazine) develop bleomycin-related pulmonary toxicity. Radiation-induced pneumonitis can also occur post-radiotherapy. BMJ Best Practice (Oct 2019) emphasised on the importance of monitoring pulmonary functions by assessing FEV1 and transfer factor for carbon monoxide (TLCO). Infertility Infertility can occur in over 50% of both male and female patients with Hodgkin's lymphoma, depending on various factors e.g. treatment dose, regimen and age of the patient. The use of chemotherapy in female patients over the age of 30 with advanced Hodgkin's lymphoma is more likely to result in a reduced ovarian function comparing to younger patients. Low doses of radiotherapy (1-2Gy) can lead to infertility in men. In terms of chemotherapy regimen, ABVD has a lower risk of infertility in this gender group. Counselling should be offered to discuss the risk of infertility and explore alternative options. Infections Patients with Hodgkin's lymphoma are often immunocompromised at the point of diagnosis. This can be caused by underlying conditions or certain genetic predispositions (e.g. human leukocyte antigen (HLA)) which impair the immune systems and make the patients more susceptible to Hodgkin's lymphoma. Neutropenia is common in patients taking chemotherapeutic regimens such as ABVD, and almost all patients receiving BEACOPP are neutropenic. Good Luck.