Disorders of adrenal gland

hi witness there's a doctor streak on some team MDS conquer now I will be talking about internal and disorders in neat and aims point of view the first important aspect that to be focused is about man syndrome okay man syndrome is of different types so the first important type is the type form so type 1 the easy way to remember is it contains three major things they are called as you can remember it as P P and P that is parathyroid hyperplasia or adenoma pancreatic neuroendocrine tumor and pituitary adenoma okay all these three P's are present in man type one symptom so apart from this in this particular individual you can see facial facial cutaneous Angie of I promise subcutaneous or visceral lipomas and this is mainly due to man one defect okay that is man one gene defect which is located on chromosome number 11 and and this particular man one syndrome is autosomal dominant you can remember that all the types of men as men one man - Man 3 and man for all are dominant you can simply remember the man syndrome all the types are dominant men are dominant right okay the next one is meant to okay men - is also called as 2a that is meant to a is also care strict with Sybil syndrome a turn cordless Sybil syndrome okay in this particular aspect you can remember it as okay previous one it is a P P and P right previous one it is P P and T whereas this particular one is you can remember it as P P and M okay M for medullary carcinoma whereas P for pheochromocytoma and other P is parathyroid hormone parathyroid hyperplasia or adenoma similarly like that of men that is ppm this the cord is ppm and it is mainly associated with the defect in the red team and it is located on the chromosome number 10 and it is autosomal dominant similarly to the type form the next comes is type 3 type 3 is also called as to be type 3 is also called as to be type 1 is the code is P P P type 2 the cord is ppm whereas type 3 the code is p.m. okay so P is pheochromocytoma am for medullary carcinoma of thyroid which is present in the 2a also then all that most of the futures are seen with megacolon or marfanoid futures all these seen okay and similarly like that of red gene similarly to that of your 2a and it is similar to that of your 2 year that is chromosome number 10 defect and all are dominant it is autosomal dominant the next one is you you you cannot found this regularly in most of the books okay but it's a newly added stuff and you have to make a note that is man thought it is also called as man X okay so these are the futures which are seen in the man x that is hyperparathyroidism pick to Tacoma pancreatic neuro endocrine tumor gonal eternal renal tumors and thyroid tumors okay can see more fair futures also in this particular individual and this is basically due to the defect of cyclin dependent kinase inhibitor which is located on chromosome con most of the features are almost similar to that of Man 1 okay the only difference between Man 4 and Man 1 is the defect oh there it is man 1 gene defect here it is cyclin dependent kinase inhibitor so you can simply add a definition for man 4s all the futures which are seen in the manual okay but if a mutation is diagnosed as cyclin dependent kinase inhibitor then you can diagnose this as meant for okay hope you're clear most of your questions with what is a common between 2a to be water which is which is the future that is seen in the sickle syndrome futures that are seen in the men for or else they can ask you the chromosome number out they can ask you to simply the mode of inheritance the mode of inheritance is common for all that is autosomal dominant a regularly asked question in the stuff that we have discussed yesterday when we were talking about thyroid gland carcinomas is which type of thyroid gland carcinoma is most commonly seen in the men syndrome is a commonly asked question the answer is the medullary carcinoma because you can see medullary carcinoma as a common future in both 2a to be okay in 2a 2b both the types you can see the medullary carcinoma has a common future fear cytochrome ax is common for it is common for 2a to pee right so such questions are most common for you in the examination and please do make a note of all the four types of this man syndrome okay so once you are done with the men's syndrome apart from this the most important aspect where a number of n number of questions I have seen is for your cytochrome okay so before that we need to talk about the eternal claim so this is an internal gland which is close in association with your kidney and I didn't gland has two major parts in it that is the cortex and the medulla and the medulla will have this particular type of cells which are mainly responsible for pheochromocytoma those cells are called as chroma findt cells ourselves are called as chromaffin cells which are the primary cause for the occurrence of pheochromocytoma okay next important aspect when you're talking about pheochromocytoma and the question that is given in 2018 neat rule of 10 is mainly associated you have other rule of 10 which we have learned in the cleft lip and cleft palate surgery that is called less rule of 10 of millet rule of 10 of Malad is cleft lip and cleft palate there is other rule of 10 which is applicable for the diagnosis or the confirmation of pheochromocytoma so what this rule of 10 says okay the rule of tenses that ten percentage of these tumors are extra at another ten percent is our bilateral means most of them are unilateral or solitary 90 percentage of them are unilateral are solitary but ten percentage are pine at ten percent is our familiar 10 percent is the current children ten percent is our malignant most of them are benign and 10% is the current man syndrome which type of men syndromes they can be seen in 2a and 2b 10% is that car with a stroke okay 10% is they occur with recurrent they are concerned with 10 chromosome okay we all learned that both 2a and 2b are associated with chromosome number 10 okay so you can add this as rule of 10 okay so all these are very very important aspect whenever this tumor occurs extra adrenaline okay the most common site of occurrence of extra adrenaline is the organ of jukkalan okay this is the most common site of occurrence of the extra Donnellan tumor of this particular type okay hope you are clear active now okay and malignancy is 10% as many know this is a benign tumor but malignancy can be seen in ten percentage of the cases okay so what happens okay you so come if you're talking about the pathology that is also cited with the a particular tumor whenever you have excess or whenever you have size increasing the size of this adrenal gland okay the edenal gland is going to produce non epinephrine as well as non internally as well as internal okay it's going to produce adrenaline and noradrenaline okay preferably it produced more non internal in than that so that's a question they can ask we just pleased to make a note so coming to the clinical features this classic trial is very very important the classic triad contains a recurrent high tech or episode of high tech they can call as diaphoresis okay and palpations the patient complaints of you can see these symptoms very rarely but the patient complaints of loss of weight that is mainly due to increasing in the energy expenditure you more energy is spent so that the patient will have loss of weight okay then the most common symptom that you see in these patients is headache okay the most common symptom that you can see is a headache and the most common manifestation okay manifestation you can see it as hypertension hypertension is the most common manifestation whereas the most common symptom is is I'll take the patient complaints of frequent headaches so in the case based question they can give you this particular Associated features because they can give you they can consider it as men's two or three syndrome they can give you the extra features or extra tumors that will present or they can ask they can tell you that a simple sign like the patient have a frequent or a severe episodic headaches which make us to deviate towards the CNS pain but it is basically this particular tumor okay you can have the loss of weight okay dude a heavy expenditure of the energy and you have several cardiac manifestations okay that can be given in the particular question about the description but in this particular mode of questions where I have seen multiple case based questions on this area the best finding that the gear is there will there really tell that upon urinary examination the patient has an increasing in BM levels or urinary you can have excess of catecholamines that are secreted okay so this is a common finding regularly or most commonly acid blindly you can go over the answer like whenever they talk about something like vm ears are increased or catecholamines have secreted then you can go straight away for this particular option so but you can see in the in the plasma you can see meta nephrons and catecholamines catecholamines meant and evidences are nothing but they are the breakdown products of the catechol immense pharma logically you can try by giving glucagon infusion to the patient whenever you give the glucagon infusion and the glucagon in this particular patient is going to secrete extra catecholamines so whenever extra katakana Mentzer and raised the patient's hypertension will be increased and the patient the blood pressure you can monitor the blood pressure so you the blood pressure will be increased so by which you can diagnosis but it's not frequently used the most commonly used are the first and second okay so coming to the other other investigations that you can make a note so as it is a soft tissue the best option is MRI you can see in this particular MRI there is the the other you can see clearly these other the these are the tumors that you can see a bilateral tumors okay so Amara is the best option either for Adele or extraordinaire and particularly you can use this in pregnancy women also with a height with a good sensitivity and specificity for identification of the disorder okay you can use CT but MRI is always the best option biopsies everything like even when you're using CT make a note you should perform this procedure without any contrast administration without any administration of contracting contrasting agent okay the main reason like even if you do biopsy or any sort of injection of contracting agent there will be there will be hypertensive crisis so which is which is not a good indication okay so biopsies and city with contrasting agent are contraindicated in these patients to avoid the hypertensive crisis so coming to the treatment plan okay so the treatment plan is just the removal okay that is adrenalectomy can be done and preferable type of item elect amis laparoscopic adrenalectomy you can do laparoscopic a little ectomy and and they can take a few few times they can consider the size of the tumor also so when you go for the laparoscopic at an elective me make sure that the tumor should be less than five centimeters in diameter okay and you can add one more point when the tumor size is more than ten centimeters in diameter the best approach of the diagnosis or the treatment is by using iodine-131 radioisotope okay you can add this particular point also okay so before going to the treatment before before you start the treatment okay so there are a few pretreatment precautions that you have to take so before starting the treatment you have to do I mean like for example if you take your eternal gland at an L gland is increasing the size so whenever your eternal gland is increasing in the size what is going to happen your nan adrenaline and or non internal norepinephrine or epinephrine values will be increased so when this nany these values are increased this has some effects on alpha 1 beta 1 and beta 2 receptors what alpha 1 effects are it is going to cause vasoconstriction it is going to cause smooth muscle construction also when it acts on beta 1 receptors beta 1 receptors is going to increase cardiac output it's going to increase the heart rate it's going to increase the stroke warnings all these are related to the CVS when it adds from the beta 2 beta 2 is mainly concerned with the smooth muscle relaxation okay smooth muscle relaxation so all these manifested manifestations has to be counted before you remove this particular gland okay how to do that okay so there are two things that you have to do before you start this particular treatment or before you're planning to remove the eternal cordis adrenalectomy which is the choice of treatment for this condition that is called as alpha adran genic blockers first you have to do alpha a transgenic blockers then once it is completed then you have to do beta a transgenic blockers so once these two are achieved then you can plan your surgery so alpha is always the first followed by beta then you can plan this particular treatment so what to be done in alpha the alpha processor will run for somewhere around 10 to 14 days okay what you have to do in this 10 to 14 days before the surgery is you have to give the drug if you knock see bench annamund this phenoxy benzene amount has to be given to the patient for 10 to 14 days and the patient should be in high sodium diet should be in high sodium diet which is greater than 5 grams per day before the surgery so by using this the alpha transgenic blockers can be done followed by once the alpha I transgenic blockage is done then you have to go for beta engineer block is by using your drug called as propanol so once alpha and genic blockage is done beta original blockage is done then you can plan for your laparoscopic I didn't elect ami surgery okay so so this is a very very important in examination point of view among the topics what I have mentioned you to complete in the in the in the particular topic of the disorders of adrenaline and parathyroid okay needle in Paris I did these two topics are very very very very important and most of them gqueues which were covered in the last few years and which which can be asked were clearly covered in this video so please try to do this to this video once or twice so that everything can be retained with a proper mind map your mind maps are very very important so that your revision will go okay all our main target is to revise everything two to three times in the last three months okay so last three months game plan is very very important where your mind maps and your note says and everything plays a very very vital role okay signing off for now dr. Steven from team MDS conquer