Addison’s Disease

The adequacy of glucocorticoid replacement (hydorcortisone, prednisone) must be assessed clinically. A WBC (white blood count) with differential (the percentage of lymphocytes and polymorphonuclear (PMN) leukocytes) can help, since patients whose replacement dose is too high tend to have a relative lymphopenia (lower percent lymphocytes), while those on excessive doses tend to have a higher percent PMN leukocytes.

Patients receiving fludrocortisone (Florinef) can be assessed for adequacy of replacement by assessment of blood pressure and serum sodium (Na) and potassium (K). Sometimes these can be normal but patients may be under-replaced and not feeling completely well; assessment of plasma renin activity may help, since it tends to be elevated in patients who are not receiving adequate fludrocortisone.

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Hypothyroidism: Diagnosis & Treatment

Google and the UCSF Department of Medicine commissioned me to write a Knol entitled “Hypothyroidism.” I’ve included some excerpts here, but you may wish to see the entire knol, which provides more details and includes sections on hypothyroidism in children and pregnant women as well as myxedema crisis.

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Journal of Clinical Oncology Paper

This paper was published in the Journal of Clinical Oncology in September, 2009. My colleagues and I describe our experience treating 50 patients with high-dose 131I-MIBG for their malignant pheochromocytoma or paraganglioma.

Download:
Phase II Study of High-Dose [131I]Metaiodobenzylguanidine Therapy for Patients With Metastatic Pheochromocytoma and Paraganglioma (PDF)

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Embolization of a large secretory paraganglioma

My colleagues and I presented two papers at the Second International Symposium on Pheochromocytoma at Queens College, Cambridge University in October 2008. We describe the successful pre-operative embolization of a large secretory paraganglioma.

Poster: Embolization of a large secretory paraganglioma (PDF)

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UCSF MIBG Protocol

Here are the files for the UCSF Phase II high-dose 131I-MIBG compassionate use protocol for treatment of patients having malignant pheochromocytomas or paragangliomas.

    In order for 131I-MIBG therapy to achieve a remission for patients with malignant pheochromocytoma, the tumors must have sufficient uptake of MIBG on diagnostic scans to allow enough131I-MIBG to enter tumor cells. Treatment doses up to 500 mCi131I-MIBG can be given without cryopreserved blood stem cells. At higher doses, there is a significant risk of severe suppression of the bone marrow. However, peripheral blood stem cell leukophoresis and cryopreservation has made it possible to treat patients with malignant pheochromocytoma more aggressively with 131I-MIBG, since peripheral blood stem cells may be collected in advance and reinfused in the event of prolonged myelosuppression following high-dose 131I-MIBG therapy.

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    Diabetes Caused By Protease Inhibitor Therapy for HIV/AIDS

    Protease inhibitors refer to a group of medications used to stop replication of the HIV (human immunodeficiency virus) retrovirus. They are very effective medications and remain an essential element in the HIV/AIDS cocktail of drugs, also known as “HAART” (Highly Active Antiretroviral Therapy). AIDS is an acronym for “acquired immunodeficiency syndrome”.

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    Acromegaly & Gigantism

    Acromegaly refers to the clinical syndrome caused by excessive growth hormone (GH) in adulthood, after the closure of epiphyses (bone growh plates). GH produces many of its effects through the stimulation of insulin-like growth factor (IGF-I), produced in the liver and at epiphyses. Symptoms of acromegaly typically include the growth of hands and feet, the growth of jaw and brow, with coarsening of facial features. Bones grow thicker and the growth of spinal bone can cause spinal stenosis and serious neurologic problems. Affected individuals are usually very sweaty, and also have increased muscle mass and reduced subcutaneous fat. Carpal tunnel syndrome is common. Diabetes and hypertension commonly occur. Untreated patients have a reduced life expectancy due to cardiovascular complications.

    Gigantism refers to excessive growth in stature during childhood and adolesence, caused by excessive growth hormone. Affected children are much taller than expected for their age. Without treatment, such children eventually develop acromegaly in addituion to their gigantism.

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    Hyperprolactinemia

    Background: Prolactin is a peptide hormone that is produced and released from the anterior pituitary. The pituitary is an autonomous prolactin factory. The pituitary will produce increased amounts of prolactin automatically unless it is inhibited from doing so by dopamine, a hormone secreted by the adjacent hypothalamus; dopamine travels down the pituitary stalk to the pituitary gland.

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    Hemoglobin A1c Testing

    Hemoglobin A1c: Hemoglobin is composed of a tetramer of globin chains; two of the chains are alpha-globin (chromosome 16). Most adults have hemoglobin that is largely comprised of two alpha chains combined with two beta chains (chromosome 11) (alpha2beta2), called HbA. The exons that encode beta-globin are adjacent to those encoding delta-globin and gamma-globin. About 2% of most adults’ hemoglobin is a variant (alpha2delta2), called HbA2. Less than 1% of most adults’ hemoglobin in fetal hemoglobin (alpha2gamma2), called HbF. A portion of HbA becomes glycosylated during its formation; a stable variety of glycosylated hemoglobin is called HbA1c.

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    Hyperparathyroidism therapy with cinacalcet

    The tiny parathyroid glands are located in the neck and are usually located adjacent to the thyroid gland, hence the term “parathyroid”. There are usually four parathyroid glands, but there can be more, and they can sometimes lie in locations away from the thyroid, sometimes elsewhere in the neck or even in the chest (mediastinum).

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    Introduction to Pheochromocytoma and Paraganglioma

    Pheochromocytomas are uncommon tumors that arise from cells of the sympathetic nervous system and may secrete catecholamines (adrenalin). Such tumors are usually found within one or both adrenal glands (90%), but may arise in other areas of sympathetic nerve cells, and are then called paragangliomas. About 10% are found to be malignant at the time or immediately after the primary tumor is discovered. Another 5% of individuals with pheochromocytomas are later found to have malignant or recurrent disease. Interestingly, about 10% of such patients do not have hypertension at all or may have hypertension only during surgical procedures or very intermittently at which time a hypertensive crisis can occur.

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    Graves’ Disease

    Graves’ disease is the most common cause of hyperthyroidism in the United States. Other causes of hyperthyroidism include multinodular goiter, toxic solitary nodules of the thyroid, and functioning thyroid cancer. Other rare causes for hyperthyroidism include TSH-secreting pituitary tumors and hCG-secreting trophoblastic tumors of the ovary or testis. Struma ovarii is another rare cause of hyperthyroidism. All of the latter conditions cause increased thyroid radioactive iodine uptake on scanning. Additional causes of hyperthyroidism include subacute thyroiditis and certain phases of Hashimoto’s thyroiditis as well as exogenous thyroid hormone intake and iodide-induced hyperthyroidism (due to kelp, amiodarone x-ray contrast).

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