11: Parathyroid disorders Flashcards

0
Q

Hyperparathyroidism- sx

A

Painful bones
Renal stones
Abdominal groans
Psychic moans

CV: HTN, short QT interval, LV hypertrophy

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1
Q

Primary hyperparathyroidism- cc

A
  • parathyroid adenoma

- parathyroid hyperplasia

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2
Q

Primary hyperparathyroidism- lab

A

An elevated PTH level with an elevated ionized serum calcium level is dx of primary hyperparathyroidism

  • Low phosphate
  • Mild increase in urinary calcium
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3
Q

Primary hyperparathyroidism- imaging

A

NOT routinely required

  • US of the neck
  • SPECT with Technetium 99
  • CT
  • Dual-energy x-ray absorptiometry (DXA): demonstrate the skeletal involvement in primary hyperparathyroidism
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4
Q

Primary parathyroidism- rx

A

-surgical excision- permanent and curative
-Bisphosphonate (i.e. alendronate): reduce bone loss
Acute hypercalcemia
-IV volume expansion with sodium chloride and loop diuretics (Furosemide) once te IV volume is restored
-Calcitonin and IV bisphosphonate: temporary prior to surgery

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5
Q

2ndary hyperparathyroidism- cc

A
  • Chronic renal insufficiency
  • VitD deficiency (osteomalacia with increased fracture and myopathy)
  • Calcium malabsorption
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6
Q

2 hyperparathyroidism- lab

A
  • Low-normal calcium and elevated PTH
  • Phosphorus: High; renal insufficiency, low; vitD deficiency
  • 25-hydroxyvitamin D: less than 20 ng/mL- deficiency
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7
Q

2 hyperparathyroidism; vitD def- rx

A

Vitamin D-2: 50,000-IU

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8
Q

2 hyperparathyroidism; renal failure- rx

A
  • Dietary phosphate restriction
  • Phosphate binders (calcium carbonate, calcium acetate, sevelamer hydrochloride)
  • Calcium supplementation should be limited
  • vitD and its analogs (calcitriol, paricalcitol)
  • Calcimimetics (cinacalcet)
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9
Q

2 hyperparathyroidism; surgical- rx

A

-Total parathyroidectomy with autotransplantation

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10
Q

2 hyperparathyroidism- Indications for surgery

A
  • Severe bone pain or fracture
  • Pruritis
  • Calciphylaxis (IV calcification leading to gangrene)
  • Extraskeletal nonvascular calcifications
  • Development of tertiary hyperparathyroidism
  • Hyperphosphatemia
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11
Q

3 parathyroidism- cc

A

Excessive secretion of parathyroid hormone after longstanding secondary hyperparathyroidism and resulting in hypercalcemia
-Change in the calcium-sensing mechanism to hypercalcemic levels

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12
Q

3 hyperparathyroidism- rx

A

Total parathyroidectomy with autotransplantation or subtotal parathyroidectomy

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13
Q

Hyperparathyroidism in pregnancy

A
  • Maternal calcium crosses the placenta resulting in chronic suppression of fetal parathyroid glands
  • Following delivery, neonatal serum calcium drops and profound **perinatal hypocalcemia ensues
  • Function returns in appro. 7-10 days
  • Total parathyroidectomy and autotransplantation in mother during the second trimester if possible
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14
Q

MEN 1 (Wermer syndrome)

A

MEN1 gene mutation

  • Parathyroid hyperplasia (MC)
  • Pituitary adenoma
  • Pancreatic islet cell tumors
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15
Q

MEN 2A

A

RET gene mutations

  • Medullary carcinoma thyroid
  • Pheochromocytoma
  • Parathyroid hyperplasia/ tumor
16
Q

Familial hypocalciuric hypercalcemia (FHH)

A

Dx: calcium-to-creatinine ratio of 99% of renal calcium resorption*

17
Q

Hyperparathyroidism-Jaw tumor syndrome (HPT-JT_

A

HRPT2 gene mutation

  • Hyperparathyroidism
  • Cemento-ossifying fibromas of the jaws
  • Wilm’s tumor
18
Q

1 hypoparathyroidism- Congenital cc

A
  • DiGeorge syndrome/Velocardiofacial syndrome (CATCH22: Cardiac, Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia with 22q deletion)
  • Kearns-Sayre syndrome: mitochondrial myopathy, ophthalmoplegia, retinal degeneration, cardiac conduction defects, primary hypoparathyroidism
  • Barakat syndrome: primary hypoparathyroidism, nerve deafness, steroid-resistant nephrosis
19
Q

1 hypoparathyroidism- sx

A
  • Chvostek sign
  • Trousseau sign
  • Prolongation of QT interval
20
Q

1 hypoparathyroidism- lab

A
  • Hypocalcemia
  • Serum albumin (low serum albumin can cause a reduction in the total, NOT the ionized, fraction of the serum calcium)
  • PTH low
  • Increased phosphate
  • Serum magnesium (severe hypomagnesemia-> PTH deficiency-> hypocalcemia: Exclude hypomagnesemia from 1 hypoparathyroidism**)
  • Alkaline phosphatase low (increased in pts with osteomalacia and rickets)*
21
Q

1 hypoparathyroidism- rx

A
  • Calcium administration
  • VitD

NOT-> PTH (not approved by FDA)*

22
Q

2 hypoparathyroidism- cc

A
  • Malignancy*
  • Hematological disorders* (MM, Lymphoma, Leukemia)
  • Paget’s disease
  • VitD toxicity
23
Q

Pseudohypoparathyroidism (PHP)

A

GNAS1 gene mutation

-lack of response to PTH

24
Q

PHP type Ia (Albright’s hereditary osteodystrophy: AHO)

A
  • Hypocalcemia
  • Short stature
  • Rounded face
  • Shortened fourth etacarpals
  • Obesity
  • Dental hypoplasia
25
Q

PHP type Ib

A
  • Hypocalcemia

- -only the kidneys affected

26
Q

Ddx: 1, 2, pseudo- hypoparathyroidism**

A

1: low concentration of PTH with a low calcium level
2: the serum PTH concentration is low and the serum calcium concentration is elevated
Pseudo: the serum PTH concentration is elevated as a result of resistance to PTH with a low calcium level