10/23/2018 1 Endocrinology and VHL: The adrenal and the pancreas LAUREN FISHBEIN MD, PHD UNIVERSITY OF COLORADO SCHOOL OF MEDICINE DIVISION OF ENDOCRINOLOGY, METABOLISM AND DIABETES DIVISION OF BIOMEDICAL INFORMATICS AND PERSONALIZED MEDICINE OCTOBER 20, 2018 Overview Definition of a hormone Adrenal gland Adrenal hormones Pancreas gland Pancreas hormones and enzymes Endocrine Glands Organs in our body that make hormones https://s-media-cache-ak0.pinimg.com/736x/5e/eb/a4/5eeba4d996ec972d35980896fe5bb654.jpg Master Gland Metabolism Energy BP/HR Food breakdown and metabolism Signals for energy usage and stores Energy BP Metabolism Female hormones Male hormones What is a hormone?
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Endocrinology and VHL: The adrenal and the pancreasThe adrenal and the pancreas LAUREN FISHBEIN MD, PHD UNIVERSITY OF COLORADO SCHOOL OF MEDICINE DIVISION OF ENDOCRINOLOGY, METABOLISM
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10/23/2018
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Endocrinology and VHL: The adrenal and the pancreas
LAUREN FISHBEIN MD, PHDUNIVERSITY OF COLORADO SCHOOL OF MEDICINEDIVISION OF ENDOCRINOLOGY, METABOLISM AND DIABETESDIVISION OF BIOMEDICAL INFORMATICS AND PERSONALIZED MEDICINEOCTOBER 20, 2018
Overview
Definition of a hormone Adrenal gland Adrenal hormones Pancreas gland Pancreas hormones and enzymes
Pheochromocytoma – leads to high blood pressure, rapid heart rate, sweating, headache, anxiety, tremors, increased blood sugar Some people with pheo have no symptoms
Flight or flight response
Pheochromocytoma (and Paraganglioma)
Pheochromocytomas (pheos) Tumors in the adrenal medulla Make adrenaline hormones About 20% of patients with VHL will develop
pheos
Paragangliomas Tumors in nerve bundles (ganglia) outside the
adrenal gland Similar to pheos Rare in patients with VHL
Petri et al British Journal of Surgery 2009
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Testing for pheochromocytoma
Blood test Plasma free metanephrines
24hr urine test Urine fractionated metanephrines
If found to have pheo, need pre-op blocking with special blood pressure medication called alpha blocker before surgery Blocks effects of high adrenaline
Insulinoma– low blood sugar, confusion, vision changes, unusual behavior, rapid heart beat, sweating, shakiness, amnesia, eating every few hours, waking up at night to eat to avoid symptoms.
Liver
Receptor
Pancreas
Insulin
Muscle
Blood sugar
Blood sugar
Blood sugar
Blood sugar
Blood sugar
Blood sugar
Glucagon
Glucagonoma – blood sugar too high causing diabetes, weight loss, blood clots and a specific rash called necrolytic migratory erythema.
Liver
Receptor
Pancreas
Glucagon
Blood sugar
Blood sugar
Blood sugar
Blood sugar
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PancreasSomatostatin
Somatostatinoma – results in dysregulation of many endocrine hormones. Lowers insulin leading to diabetes. Slows GI motility which can lead to gallstones, intolerance to fat in the diet and leads to fatty diarrhea.
Receptor
Pancreas
Somatostatin
Nerve
glucagoninsulin
Pituitary picture from https://s-media-cache-ak0.pinimg.com/236x/3d/45/12/3d4512b044af3b0e5877a78499114d4e.jpg
Decrease acid Slow
motility Secrete pituitary
hormones
Vasoactive Intestinal Polypeptide(VIP)
VIPoma – causes huge amounts of very watery diarrhea leading to dehydration, low potassium and chloride
Liver
Receptor
Pancreas
VIP
glycogen
Energy
Pancreasbicarb
Majority of PNETs in VHL are non-functional
So why am I talking about the hormones?
A pancreas that is not functioning well or is absent due to surgery, may make too little hormone and enzymes We can replace these with medications
Abnormal functioning pancreas
Insulin is the main hormone of concern when absent Insulin deficiency causes diabetes mellitus We can replace insulin to control diabetes
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Insulin
Liver
Receptor
Pancreas
Insulin
Muscle
Blood sugar
Blood sugar
Blood sugar
Blood sugar
Blood sugar
Blood sugar
Abnormal functioning pancreas
Absent exocrine pancreas is another concern Absent enzymes cause bloating and discomfort
because food cannot be digested well We can control the symptoms with giving back the