Feedback control Endocrinology Update...Best in young patients with small gland and mild disease About 30% of patients have “remission” after 6 months to several years >60% relapse

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Endocrinology Update

Robert J. Rushakoff, MDProfessor of Medicine

University of California, San [email protected]

Feedback control

Golden Rule of Endocrinology

If overproduction is suspected, try to suppress production.

If underproduction is suspected, try to stimulate production.

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Pituitary Hormones and Their FunctionHormone Increased by Decreased by Excess Deficiency Notes

ADH Thirst, high serum osmolality

Low serum osmolality, low serum K+

SIADH DI

ACTH CRH, Stress High cortisol Cushing’s syndrome

Adrenal insufficiency

Diurnal variation (peak at 3–4 a.m.)

TSH TRH High T 4 and/or T3

Hyperthyroidism Hypothyroidism

LH/FSH GnRH Gonadal sex steroids

Hypogonadism In men, inhibin inhibits FSH

GH GHRH, hypoglycemia,dopamine

Somatostatin Childhood: gigantism Adulthood: acromegaly

Child: short statureAdult: poor sense of well-being

Prolactin Pregnancy, nursing, TRH, stress

Dopamine Galactorrhea, hypogonadism

Inability to lactate Under tonic inhibition by hypothalamic dopamine


ADH Thirst, high serum osmolality Low serum osmolality, low serum K+

SIADH DI

ACTH CRH, Stress High cortisol Cushing’s syndrome Adrenal insufficiency Diurnal variation (peak at 3–4 a.m.)

TSH TRHHigh T4 and/or T3

Hyper-thyroidism

Hypo-thyroidism

LH/FSH GnRH Gonadal sex steroids Hypogonadism In men, inhibin inhibits FSH




Prolactin Pregnancy, nursing, TRH, stress Dopamine Galactorrhea, hypogonadism Inability to lactate Under tonic inhibition by hypothalamic dopamine



SIADH DI

ACTHCRH, Stress

High cortisol

Cushing’s syndrome

Adrenal insufficiency

Diurnal variation (peak at 3–4 a.m.)

TSH TRH High T4 and/or T3 Hyperthyroidism Hypothyroidism





Prolactin Pregnancy, nursing, TRH, stress Dopamine Galactorrhea, hypogonadism Inability to lactate Under tonic inhibition by hypothalamic dopamine



SIADH DI


TSH TRH High T 4 and/or T3 Hyperthyroidism Hypothyroidism


GHGHRH, hypoglycemia,dopamine

Somatostatin

Childhood: gigantism Adulthood: acromegaly


Prolactin Pregnancy, nursing, TRH, stress

Dopamine Galactorrhea, hypogonadism Inability to lactate Under tonic inhibition by hypothalamic dopamine

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SIADH DI


TSH TRH High T 4 and/or T3 Hyperthyroidism Hypothyroidism





ProlactinPregnancy, nursing, TRH, stress

DopamineGalactorrhea, hypogonadism

Inability to lactate

Under tonic inhibition by hypothalamic dopamine

- SOMATOSTATIN

Hypothalamus

TRH +PIT

TSH +-

-

T4

T3

Hormone Action

TSH (+)

T4, T3 (-)

Thyroid HormoneAction

ThyroidPITUITARY

T4, T3(+)

Hormone ActionThyroid Failure

↑↑TSH (+)

T4, T3 (-)

Thyroid HormoneAction

ThyroidPITUITARY

T4, T3(+)

X

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Hormone ActionPrimary Hyperthyroidism

↓ ↓ TSH(+)

↑ T4, T3

(-)Excess Thyroid HormoneAction

ThyroidPITUITARY

↑ T4, T3

Thyroid Function Tests

� Total Thyroid Hormone�T4

�T3

� Free Thyroid Hormone�direct and indirect measurement�FT4, FT3

� Tests for Thyroid hormone binding protein�T3U�TBG

TSH

EuthyroidPrimary

HyperthyroidPrimary

Hypothyroid

normal

Radioactive Iodine Uptake

� 123I given orally

�Gamma counter over area of thyroid measures radioactivity at 24 hours

�Useful for differential diagnosis of thyrotoxicosis

�NOT A TEST FOR ASSESSMENT OF THYROID FUNCTION

�Dependent on iodine intake

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Radioactive Iodine Scanning

� Image of thyroid obtained after the administration of RAI

�NOT A TEST FOR ASSESSMENT OF THYROID FUNCTION

�Useful for when thyrotoxic nodular disease is suspected.

Thyroid Function and Oral Contraceptives

Serum T4

Hyperthyroid Euthyroid

T3 Uptake

Free T4

TSH

T3 Uptake

Patient ASBPPatient TDF

Serum T4

Euthyroid

Free T4

TSH

Anabolic Steroids

Patient SF

FH + for mother with thyroid disease

Medications: none

Exam: Thin female, hyperactive

Pulse regular at 120. BP: 98/60

HEENT: + stare, + lid lag at 98mm measure 17mm bilat

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Patient SF

Neck: thyroid enlarged 2X, beefy, no nodules

Heart: + Means-Lerman systolic scratch (rubbing together of normal pleural and pericardial surfaces)Ext: fine tremor, no rash

Do you suspect that the patient

has hyperthyroidism?

Symptoms of Hyperthyroidism

� Palpitations� nervousness� fatigue� hyperkinesia� hyperdefication� sweating

� Heat intolerance� weight loss (with

increased appetite)� Neck swelling� Neck pain� Changes in eyes� decrease in menstrual

periods

Symptoms of Hyperthyroidism

� Palpitations� nervousness� fatigue� hyperkinesia� hyperdefication� sweating

� Heat intolerance� weight loss (with

increased appetite)� Neck swelling� Neck pain� Changes in eyes� decrease in

menstrual periods

Patient SF

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Signs of Hyperthyroidism

� Tachycardia � Lid lag� Stare� exophthalmus

(Graves’ disease)� Tremor� Thyroid enlargement

� Onycholysis (separation of the nail from its bed)

� brisk reflexes� Dermopathy

(thickened skin, most often pretibial)

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Is the patient

thyrotoxic?FT4 is 36 (nl 12-24)

TSH is 0 (nl 0.5-5)

Laboratory Tests for Thyrotoxicosis

TSH

FT4

Primary thyrotoxicosis

TSH

FT4

Secondary thyrotoxicosis

Laboratory Tests for Thyrotoxicosis

TSH

FT4

? T3 toxicosis

TSH

FT4

FT3

T3 toxicosis

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Laboratory Tests Not Usually Needed for Diagnosis

�TSI

�Thyroid Antibodies

Causes of Thyrotoxicosis

�Common Causes�Graves’ Disease

autoimmune disease - B lymphocytes produce TSIFemale:Male 5:1Peak age 20-40

�Toxic Multinodular Goiter (TMNG)�older patients with history of multinodular goiter�may be precipitated by iodine


�Common Causes�Toxic Nodule (Plummer’s Nodule)

Single autonomous nodule (>3cm)Age usually > 40

�Subacute thyroiditis�Young patient�history of malaise, neck pain, viral infection�thyroid may be slightly large and tender


� Less Common Causes�Jod Basedow

iodine induced (usually in patient with underlying goiter)

�Silent Thyroiditis� factitious

� excessive thyroid hormone ingested, usually for weight control

� Most often someone in medical field

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�Less Common Causes�hypothalamic-pituitary�post-partum

“postpartum depression”followed by hypothyroidism

�molar pregnancyhigh levels of hCG

�Struma Ovariiteratoma of ovary containing thyroid tissue

Tests for Differential Diagnosis

Graves’ Disease

Subacute Thyroiditis

TSH FT4 24h RAI uptake

Graves’ gland “beefy”Thyroiditis gland hardESR high in Subacute thyroiditis


Graves’ Disease

Subacute Thyroiditis

TSH FT4 24h RAI uptake

Graves’ gland “beefy”Thyroiditis gland hardESR high in Subacute thyroiditis


TMNG

Toxic Nodule

TSH FT4 FT3 24h RAI uptake

TMNG : feel a multinodular goiter, scan shows hot spotsToxic nodule: palpate a single large nodule, scan shows single hot spot

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Graves’ Disease Treatment

Antithyroid Drugs

Radioactive iodine

Surgery

Antithyroid Drug Therapy

�Best in young patients with small gland and mild disease

�About 30% of patients have “remission” after 6 months to several years

�>60% relapse


� Propylthiouracil (PTU)� Initail dose 150-200 mg tid

� Methimazole (tapazole)� Initial dose 20-30 mg qd

� Side effects� rash in 5% - can often just treat with antihistamine�agranulocytosis in <0.5% - patients told to stop drug

and get WBC if fever and sore throat


1. Start Tapazole 20-30 mg per day2. Start propranolol 20-40 mg tid and have patient titrate to maintain pulse 60-90. Large doses may be required.

1. Follow FT4 monthly lowering Tapazole to maintain in normal FT4. TSH will remain suppressed for months and will not be useful.

2. Titrate propranolol off as patient becomes euthyroid.

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Surgical Treatment

�Treatment of choice for patients with very large goiters

�Risk for hypoparathyroidism

�Risk for recurrent laryngeal nerve injury

�Surgeon will try to leave tissue so patient remains euthyroid

�Risk for late recurrence

Radioactive Iodine Treatment

�For most patients treatment of choice�Contraindicated in pregnancy, if

pregnancy anticipated in next year, and possibly if exophthalmus present (very controversial).

�Currently, usual treatment is to ablate thyroid, then have patient take thyroid hormone

Treatment for Subacute Thyroiditis

�Treatment of symptoms

�Treat with beta blocker titrated to lower pulse

�Antithyroid medications of no use

�ASA or NSAIA for pain� toxic state should resolve in 2-3 months

Treatment for Subacute Thyroiditis

�After toxic period, may have transient hypothyroidism (in some patients permanent).

�May choose to just watch patient during this time and most will be euthyroid in another 2-4 months.

�May start on levothyroxine and then stop in a year to see if needed long term

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Patient LL

Patient is a 21 year old female who is preparing for finals. She complains of mild fatigue and heavy menstrual periods. She is on no medications (including birth control pills). She has gained 15 lbs in past 2 years. Patient’s sister takes thyroid hormone.

Physical exam is normal.

Do you suspect that the patient

has hypothyroidism?

Symptoms of Hypothyroidism

�Fatigue

�Cold intolerance

�Weight gain�constipation

�heavy, frequent menstrual periods

�muscle cramps

Symptoms of Hypothyroidism

�Fatigue�Cold intolerance

�Weight gain�constipation

�heavy, frequent menstrual periods�muscle cramps

Patient LL

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Signs of Hypothyroidism

�Dry skin

�puffy face and hands

�hoarse�slow reflexes

Signs of Hypothyroidism

�Dry skin

�puffy face and hands

�hoarse�slow reflexes

Patient LL

Is the patient hypothyroid

?TSH is 35 (nl .5-5).

Laboratory Tests for Hypothyroidism

TSH

FT4

Primary hypothyroidism

TSH

FT4

Secondary hypothyrodism

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Laboratory Tests for Hypothyroidism

TSH

FT4

Subclinical hypothyroidism

Clinical significance not clear.

Patients with TSH >20 or + thyroid antibodies have >80% chance to become clinically hypothyroid within 5 years

Etiology of Hypothyroidism

�Primary�Hashimoto’s Thyroiditis

automimmune+/- goiter

�After RAI or surgery for Graves’ disease�subacute thyroiditis�inborn errors of thyroid hormone synthesis

Secondary�hypothalamic or hypopituitarism

Thyroid Hormone Preparations

�Levothyroxine (T4) �most physiologic�treatment of choice�daily dose�t1/2 1 week �average dose 0.125 mg/d


�Liothyronine (T3)�tid dosing�not physiologic �bypass normal T4 to T3 conversion�t1/2 1 day

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�Desiccated thyroid�potency variable�variable T4 and T3 levels�monitoring difficult �bypass normal T4 to T3 conversion�80% of patients on this preparation shown not

to require any thyroid hormone replacement

Monitoring Thyroid Hormone Replacement

Goal is for normal TSHMinimal time after any change in thyroid hormone dose before rechecking TSH level is 6-8 weeks. It takes this long to reach steady state. More frequent testing will be not only a waste of money but potentially misleading.Decreased Absorption:– Drugs, supplements: calcium, iron, soy,

Cholestyramine– Decreased gastric acid: H. pylori infection,

omeprazole

Monitoring Thyroid Hormone Replacement

TSH

FT4

Subclinical hyperthyroidism

Associated with decreased bone density.Associated with atrial arrhythmias in older patients.

Patient needs decrease in thyroid hormone dose.

Thyroid Nodules

� In experienced hands, thyroid US can be first diagnostic test

�Fine needle aspiration alternative first diagnostic test

�Experienced pathologist of key importance

�Thyroid radioactive scans not helpful to determine if nodule is benign or malignant

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HypopituitarismAlthough primary organ failure is more common, hypopituitarism is in the differential diagnosis in cases of hypothyroidism, hypoadrenalism and hypogonadism

GH, gonadotrophins, TSH, ACTH, Prolactin (loss in this order)

ADH deficiency only if posterior pituitary involved

Etiologies – 9 I’sInvasive – tumors, metastasisInfarction – Sheehan’s, apoplexyIatrogenic –radiation, surgeryInfiltrative – sarcoid, hemochromatosis, histiocytosis XInjury – head trauma (also can see DI)Immunologic – lymphocytic hypophysitisInfections – TB, syphilis or fungiIdiopathic Isolated – Kallman’s – GnRH deficiency with anosmia – X linked dominant – decreased expression of KAL 1

Hypopituitarism: Dx and Tx

In general, treat underlying cause

Central hypothyroidism– TSH is not reliable for screening or for

monitoring– Diagnosis depends on low Free T4– Tx: LT4 therapy, but do not start until

adrenal insufficiency has been ruled out

Hypopituitarism: Dx and Tx cont’d

Central adrenal insufficiency– Aldosterone secretion intact so no

hyperkalemia– Hyponatremia due to decreased GFR &

inability to excrete water load (+ increased ADH secretion +/- hypothyroidism)

– Dx: cosyntropin stimulation test (random cortisol or ACTH level not useful)

– Tx: hydrocortisone 20-30 mg/d, 2/3 in the morning and 1/3 in the evening.

Prolactin deficiency – inability to lactate

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Pituitary tumors

�Microadenomas are < 1cm

Macroadenomas are >1cm

Types of tumors– 60% prolactinomas– 20% GH producing– 10% ACTH producing– 10% non-functioning – Rare: TSH, LH/FSH or α-subunit producing

Pituitary tumors - Presentation

Symptoms/Exam:–Neurological syx (h/a, visual field

cuts, nerve palsies)–Hormonal excess or deficiency –Incidental discovery on imaging

study up to 10% general population have pituitary incidentalomas

Pituitary tumors - Evaluation

Once tumor is identified, must determine if functional or not:– TSH, FT4– Prolactin– ACTH, cortisol

Cosyntropin stimulation test if suspect deficiency24 hr urinary cortisol or dexamethasone suppression test if suspect excess

– LH/FSH + testosterone in men

Pituitary tumors - TreatmentSurgery: transsphenoidal approach is successful in approximately 90% of patients with microadenomasMedical: – Dopamine agonists for prolactinomas– Somatostatin analogues for GH producing tumors

Radiotherapy after surgery and/or medical therapy fails or poor surgical candidates:– Conventional XRT or– Gamma knife

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Hyperprolactinemia Hyperprolactinemia

Physiologic:– Pregnancy (prolactin

can reach 200 ng/ml in 2nd trimester)

– Lactation– Nipple stimulation– Exercise

– Coitus– Stress– Sleep

Pathologic:– Pituitary tumors –

mostly microadenomas

– Pituitary stalk lesions– Hypothalamic lesions– Chest wall /spinal cord

lesions– Hypothyroidism – Chronic kidney

disease– Cirrhosis

Hyperprolactinemia Cont’dPharmacologic:– Estrogen– Dopamine antagonists (phenothiazines,

haloperidol, risperidone, metoclopramide, methyldopa, opioids, amoxapine)

– Monoamine oxidase inhibitors– Cimetidine and Ranitidine– SSRI’s– Tricyclic antidepressants – Verapamil– Liquorice

Hyperprolactinemia: Syx/exam

Womengalactorrhea, amenorrhea or oligomenorrhea with anovulation and infertility in 90%Amenorrhea: 18% prolactinomaGalactorrhea: 37% ProlactinomaBoth: 75% ProlactinomaMicroadenoma: 64%

Menimpotence, decreased libido, galactorrhea (very rare)91% macroadenoma

Bothlocal tumor extension symptoms - headache, visual field cuts, hypopituitarism

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Hyperprolactinemia: DiagnosisElevated prolactin:– PRL >200 ng/ml virtually diagnostic of

prolactinoma– PRL 100-200 usually prolactinoma– PRL 20-100 may be microadenoma but

exclude other causes

Normal TFT’s and negative pregnancy test

MRI of pituitary

Hyperprolactinemia: Treatment

Medical – 1st line in most cases– Dopamine agonists: bromocriptine or

cabergolineSE: nausea, fatigue, nasal stuffiness and postural hypotension

– Restores fertility and gonadal function in most patients

– Leads to tumor shrinkage– During pregnancy, risk of growth is <5% for

microprolactinomas and 20-40% for macroprolactinomas

Surgical: – For patients intolerant or resistant to

medications– If need urgent decompression of the sella

turcica for visual field defects unresponsive to trial of dopamine agonists

Hyperprolactinemia: Treatment Pituitary Tumors: GH Tumors

80% are macroadenomas

15% co-secrete prolactin

Very rarely (<1% cases), acromegaly due to ectopic GH or GHRH production– Lung CA, carcinoid, or pancreatic islet cell

tumors

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Pituitary Tumors: GH TumorsClinical features due to excess IGF-I and mass effect of the tumor.– Hypertension– Glucose intolerance or DM– Increased colonic polyp frequency– Soft tissue proliferation: coarsening of

facial features, hand and feet enlargement– Sweaty palms and soles– Carpal Tunnel Syndrome– Hypogonadism– Visual field cuts and h/a’s

Pituitary Tumors: GH Tumors

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Gigantism

Identical twins, 22 years old, excess GH secretion

Pituitary Tumors: GH TumorsDiagnosis:– Elevated IGF-1 is the hallmark– Dx: oral glucose tolerance test

100 g glucose given60 minutes GH<1 ng/ml rules out acromegaly

– Random GH level not helpful

Therapy:– Surgery: curative in 60-80% – Radiation therapy: adjuvant therapy– Medical:

Octreotide LAR (SS analog)Pegvisomant (GH receptor antagonist)

Adrenal Insufficiency: EtiologiesPrimary AI:– Autoimmune– Metastatic malignancy & lymphoma– Hemorrhage: pregnancy, anticoagulation,

antiphospholipid antibody syndrome– Infiltrative disorders: amyloid, hemochromatosis– Drugs: ketoconazole, metyrapone,

aminoglutethimide, trilostane, mitotane, etomidate– Rare: congenital adrenal hyperplasia, adrenal

leukodystrophy

Secondary AI:– Iatrogenic: glucocorticoids & anabolic steroids– Pituitary or hypothalamic tumors

Vague syx:– Weakness– Fatigue– Anorexia– Weight loss– Nausea– Vomiting– Diarrhea– Unexplained abdominal pain– Postural lightheadedness

AI: Clinical Features

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Hyperpigmentation characteristic of primary adrenal insufficiency

AI: DiagnosisLabs: hyponatremia, hyperkalemia, eosinophilia, mild metabolic acidosis, hypercalcemia

Step 1: confirm diagnosis– Any random cortisol ≥ 18 µg/dl rules out AI– Cortrosyn stimulation test

Obtain baseline cortisol and ACTHInject Cortrosyn 250 µg IM or IVCheck cortisol level 45-60 minutes laterNormal if post stimulation cortisol ≥ 18 µg/dl

Step 2: distinguish 1o from 2o

Step 3: evaluate cause (CT abdomen or MRI pituitary)

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AI: Treatment

Hydrocortisone 20-30 mg/day– 2/3 in the morning– 1/3 in evening

Stress doses:– Minor stress – double usual dose– Major stress: 50 mg IV q 6-8 hrs, and taper

For 1o AI, also need:– Fludrocortisone 0.05-0.1 mg/day

Cushing’s SyndromeExogenous steroids: #1 causeEndogenous:– Cushing’s disease (70%): due to ACTH

secretion from pituitary adenoma– Ectopic ACTH (15%): from non pituitary

tissue making ACTHSmall cell lung CA most commonBronchial carcinoids

– Adrenal (15%):AdenomaCarcinomaNodular adrenal hyperplasia

Cushing’s Diagnosis

ACTH

Adrenal Pituitary or Ectopic

CT Adrenals MRI pituitary

Petrosal Sinus ACTH

Ectopic ACTH-producing tumor Pituitary source

Low (<5) High (>10 pg/ml)

+Central

_

Peripheral

24 h Urine Cortisol

+

_

Normal

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Primary Aldosteronism (PA)

Accounts for 0.5-10% cases htn

Results from autonomous aldosterone prdctn, due to:

Idiopathichyperaldosteronism (1/3)

Adenoma (60-65%)

Glucocorticoidsuppressible (~1%)

Cancer (<1%)

Primary Aldosteronism: Features

Hypertension

Hypokalemia– May be absent– Exacerbated by diuretics

Paresthesias if severe hypokalemia

Mild alkalosis on laboratories

Primary Aldosteronism: Dx

Screening:– Must first replete potassium– Plasma aldosterone (PA)/ Plasma Renin

Activity (PRA) ratio >20-25 � suggestive

Confirmatory test:– Salt load: 1 g NaCl tid for 3 days– 24 hr urine aldosterone>12 mcg/24 hr with

concomitant 24 hr urine Na>200 mmol/d

Primary Aldosteronism cont’d

CT– Unilateral lesion– Bilateral Hyperplasia, or no lesion seen:

Adrenal vein sampling – measure cortisol and aldosterone to lateralize source of aldosterone excess

Management:– Unilateral lesion � resect– Bilateral hyperplasia or no lesion � medical

rx with spironolactone or eplerenone

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Pheochromocytoma

Rare tumorsProduce epinephrine and/or norepinephrineSymptoms:– Episodic throbbing in the chest, trunk and head– Headaches– Diaphoresis– Palpitations– Tremor, anxiety, nausea, vomiting, fatigue– Abdominal or chest pain– Weight loss– Cold hands and feet

Pheochromocytoma cont’d

In ¼ of cases, hypertension is episodic

Orthostasis usually present

Rule of 10’s:– 10% normotensive– 10% occur in children– 10% are bilateral– 10% are malignant– 10% are extra-adrenal (called

paragangliomas)

Pheochromocytoma cont’d

First – make BIOCHEMICAL dx:– 24-hr urinary metanephrine and

normetanephrine or plasma free metanephrine (levels usually 2-3 x normal)

If biochemical diagnosis made, localize:– CT or MRI of the adrenal– If CT or MRI negative, can use 123I-MIBG to

localize extra-adrenal pheo and mets

Adrenal Incidentalomas

Found in ~10% of autopsies

Prevalence increases with age

2 questions for evaluation:– Is lesion functioning or not?– Is lesion benign or malignant?

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Incidentaloma: is it functional?In all patients:

Screen for pheochromocytoma:– 24 hr urine for metanephrines or Plasma

metanephrines

Screen for subclinical Cushing’s– 1 mg overnight dexamethasone suppression test– 24 hour urine cortisol

In patients with hypertension:Screen for primary aldosteronism:– PA/PRA ratio

Incidentaloma: Benign or not?

More likely to be BENIGN if:

<4 cm

Low density on unenhanced CT (<10 HU)

It contains fat (suggests myelolipoma)

Incidentaloma: Management

Yes No

Functional?

Size on CT

>4 cm <4 cm

Adrenalectomy

Re-image at 6, 12 and 24 mos

No further f/u if no change in size

Increase in size >1 cm

Adrenalectomy

Hypercalcemia

PTH

Primary HPTFHHLithium

HIGH

LOW

↑ PTHrPSolid tumorsT cell lymphoma

Malignancy

−1,25 Vit Dlymphoma Local osteoclastic

LesionsMyeloma, leukemiaLymphoma

OthersMilk-alkali syndromeImmobilization

EndocrineThyroid, adrenal,Pheo, VIPOMAAcromegaly

Granulomas↑ 1,25 Vit DSarcoid, TB, Cocci,Histo, pulm eosinophilia

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Primary Hyperparathyroidism

� PTH, � Ca, � Phos

0.4% women over 60; 2-3Xs the rate in men

Single adenoma 80 %, rest hyperplasia

Associated with MEN1, MEN 2A, isolated familial

Tx: Surgical if indicated

Primary HyperparathyroidismTreatment: – Surgery if

Serum Ca >1.0 mg/dl above NL24 hr urine Calcium>400 mg/dCrCl reduced by > 20%

BMD T-score <-2.5 at any siteAge <50 yrs

– If no surgery, follow with: Ca 2x/yr, Cr q yr BMD q yr

– Medication:Cinacalcet: oral calcimimetic agent decreasing PTH and calcium

Familial Benign HypocalciuricHypercalcemia (FBHH)

Mutation in calcium sensorAutosomal dominant inheritanceLifelong asymptomatic hypercalcemiaUrine Ca < 50 mg/24 hrCalcium creatinine clearance ratio < 0.01

urine Ca X Serum Creatserum Ca X Urine Creat

No need for treatment!

Documents

Feedback control Endocrinology Update...Best in young patients with small gland and mild disease About 30% of patients have “remission” after 6 months to several years >60% relapse