Disorders of Parathyroid

Glands

Dr. Akash Gupta

Sec. DNB ENT
BPS GMC, Khanpur Kalan
 Parathyroid gland

 Parathyroid consists of four small glands which are paired and

located behind the thyroid gland in the neck.
 They produce and release parathyroid hormone which is involved in
metabolism of calcium and phosphorus.
 They are produced by the chief cells
Calcium homeostasis
 Disorders of parathyroid
gland

Hyperparathyroidism Hypoparathyroidism
Hyperparathyroidism is defined by elevated serum PTH
(10-65 pg/ml)
And is the commonest pathological condition affecting the PTG

Hyperparathyroidism

Primary Secondary Tertiary

Primary Hyperparathyroidism
Over production of PTH due to intrinsic abnormality of one
or more glands causing elevated serum Calcium and
depressed serum phosphate
Causes
 PTG Adenoma 85%
 PTG Carcinoma 1%
 PTG Hyperplasia 14%
• Sporadic
• MEN 1
• MEN 2
• Familial isolated Hyperparathyroidism
• Familial Hypocalciuric hypercalcemia
Secondary Hyperparathyroidism
Compensatory hyperplasia of PTG due to decreased Serum Calcium
usually resulting in Normocalcemia

Causes
 Chronic Renal failure
 Malabsorption
 Vitamin D deficiency
 Renal Tubular acidosis
Tertiary Hyperparathyroidism
Autonomous PTG hyperfunction following secondary
hyperparathyroidism

Causes
 Any cause of secondary hyperparathyroidism
 Primary Hyperthyroidism

Clinical Presentation

 Affects all age groups with a peak incidence between 50 and 60 years of age.
 Female: Male ratio of 3:1
 Typically patients have elevated levels of serum calcium concentration on
routine health screening or an unrelated health problem.
 Band keratopathy a former hallmark of classic primary hyperparathyroidism
resulting from deposition of calcium-phosphate crystals in the cornea is rarely
seen.
 Osteitis fibrosa cystica-salt and pepper skull, cystic bone disease of clavicle,
subperiosteal bone resorption of digits, cortical erosions. occurs in 2% of
patients.
Osteitis fibrosa cystica
Clinical consequences of primary
hyperparathyroidism
Bone Destruction Hypercalcemia Hypercalciuria
osteopenia Peptic ulcer disease urolithiasis
osteoporosis pancreatitis nephrocalcinosis
Bone deformities and Constipation, nausea, Nephrogenic diabetes
fractures vomiting or loss of appetite insipidus
Osteitis fibrosa cystica, brown Polydipsia and polyuria
tumours
Renal failure, cardiovascular
features: hypertension,
arrythmias,
Tiring easily or weakness
Neuropsychiatric disorders
 Cortex thinned out, marrow contains
increased amounts of fibrous tissue
Reduction in bone mineral density with foci of hemorrhage and cyst
formation
Osteitis fibrosa Cystica

Skeletal changes in
PHPT

Brown tumours of
hyperparathyroidism
Aggregates of osteoclasts, reactive
giant cells and hemorrhagic debris
 Band Keratopathy
deposition of calcium in the sub epithelium, bowmans layer and anterior
stroma
Symptoms associated with Hypercalcemia
“Renal Stones, Painful Bones, Abdominal Groans, Psychic
Moans, Fatigue Overtones”

 Cardiovascular: Angina, dyspnea, palpitations, syncope

 Gastrointestinal: Anorexia, constipation, epigastric pain, nausea,
vomiting
 Neuromuscular: Anxiety, confusion, depression, fatigue,
forgetfulness, impaired vision, insomnia, lethargy, weakness
 Renal: Polydipsia, Polyuria, renal colic
 Skeletal: Arthralgia, bone pain, fractures
Parathyroid localization techniques

 Radiology based studies

Ultrasonography
Computed Tomography
Magnetic Resonance Imaging

 Nuclear Medicine based

Sestamibi scan
Tetrofosmin
PET
 Invasive procedures
FNAC
Parathyroid arteriography
Selective venous sampling
Adenoma in left inferior gland, visible in Early phase (A) and delayed phase (B) of planar
Delayed phase of planar Tc99m sestamibi Tc99m sestamibi scan showing high uptake in
Scan. Parathyroid gland consistent with adenoma.

Mibi: methoxy isobutyl isonitrile

Normocalcemic primary
hyperparathyroidism(a variant of PHPT)
 Increased PTH
 Normal serum total and ionized calcium concentration
 Features of PHPT may be present (eg. Low BMD)
 All secondary causes for hyperparathyroidism must be ruled out

 Normocalcemic PHPT is considered to be an early form of asymptomatic PHPT

Humoral hypercalcemia of malignancy

 Mediated by the production of Parathyroid hormone related peptide

 PTHrP acts on RANKL

SCC Lung
Stimulation of osteoclasts Urinary tract cancer(RCC)
Breast cancer
Non hodgkins lymphoma
Ovarian cancers
Resorption of calcium from the bones
And release into the blood
 Parathyroid crisis

Dehydration(hypercalciuria)

CNS dysfunction
Confusion Serum GI Dysfunction
Dizziness calcium > Constipation
Paralytic ileus
Nausea, vomiting 15mg/dl

Bradycardia( QT
shortening)
 Treatment for primary
Hyperparathyroidism

Parathyroidectomy Pharmacotherapy
 Surgery is suggested as per the new guidelines
of 2009 for those meeting the following critera
1. Serum Calcium >1.0 mg/dl above the reference limit
2. Creatinine clearance < 60cc/min
3. Markedly reduced bone density at any site (T score < 2.5
or fragility fracture)
4. Age < 50 years
 Preoperative preparation

 Vocal cords should be assessed preoperatively

 High calcium levels preoperatively may require treatment with
 Hydration
 Diuresis
 Steroids (prednisolone 20 mg TID for 5 days before surgery)
 100 mmol phosphate infusion before in 6 hours
 200 units calcitonin subcutaneous injection for 5 days twice daily before surgery
 Bisphosphonate- etidronate disodium 7.5 mg/kg daily as slow IV infusion for 3 days;
Mithramycin 25 mcg/kg as single dose
Surgical techniques

 Minimally invasive technique

 Bilateral cervical exploration- ideal for multigland disease
 Median sternotomy
 Video assisted parathyroidectomy
 Endoscopic parathyroidectomy
 Remedial parathyroidectomy- done for persistent HPT through lateral approach between
anterior margin of sternocleidomastoid and strap muscles (feind technique)
 Subtotal parathyroidectomy- indicated in hyperplasia or secondary HPT, wherein 3 1/2 are
removed , retaining ½ of the gland
• Surgical removal of the glands and implantation of fragments of the gland in forearm
muscle (brachioradialis) or neck (sternomastoid)
 If its carcinoma, additional hemithyroidectomy with post operative radiotherapy is
required
 When all four glands are diseased, transcerival thymectomy is also added along with total
parathyroidectomy to reduce persistent and recurrent disease.
 Hungry bone syndrome

 Occurs after parathyroidectomy because of sudden cessation of bone breakdown causing

bones to absorb calcium, magnesium and phosphorus rapidly.
 Hypocalcemia, hypophosphataemia, hypomagnesemia and
hyperkalemia are the typical features
 Bone specific ALP continues to rise in first few weeks indicating increased
bone reconstruction
 Supplementation of Vit D and elemental calcium is required for 6 months
 Post operatively calcitriol with 2 gm calcium supplement is required
 Post operative hypocalcemia

Features of hypocalcemia tetany

 Circumoral paresthesia, paresthesia of neck, fingers and toes
 Twitching and weakness of tongue muscles, hand, foot and digits- carpopedal
spasm

 Fingers are extended except at MCP joints and thumb is

strongly adducted(obstetrician’s hand)
 Stridor and difficulty in breathing due to paralysis of respiratory
muscles
 Chvostek’s sign- tapping above the angle of the jaw to stimulate branches of
facial nerve causes twitching of angle of mouth and eye lids

 Trousseau’s sign- applying

Sphygmomanometer to arm and
Inflating pressure >200mm hg for 3
Minutes can demonstrate
Carpal spasm
 Generalised weakness and twitch
over the body in severe cases mimic
convulsions
Management for Hypocalcemia

 Serum calcium will be less than 7 mg/dl

 IV Calcium Gluconate 10 ml of 10% solution administered in 50 ml NS over 5

minutes; if hypocalcemia continues, next infusion with 900mg of 10% calcium
gluconate in 1 litre of 5% glucose or 0.9% NS over 24 hours
 Daily ECG with QTc monitoring
 Daily Serum electrolytes
 Later oral calcium 1 gram TID with vitamin D supplementation (1-3 mcg
daily)
 Follow up at regular intervals with serum calcium monitoring
Drug treatment
 Oral phosphate:
lowers serum Calcium by up to1 mg/dl
Side effects include GI intolerance, possible increase in PTH levels
This is not advisable as a long term treatment for primary hyperparathyroidism

 Bisphosphonates:
Anti resorptive agent with overall effect of reducing bone turnover.
Although they do not affect PTH secretion directly, bisphosphonates could reduce serum and
urinary calcium levels.
Alendronate might be useful in patients with low bone density in whom parathyroid surgery is
not to be performed.
 Estrogen therapy and Selective estrogen receptor modulators:
In post menopausal women with primary hyperparathyroidism, use of raloxifene has shown
reduction in total serum calcium levels though PTH levels do not change.
 Calcimimetic agents
Calcimimetics act on parathyroid cell calcium sensing receptor, mimicking the effect of
extracellular calcium which leads to activation of the receptor and subsequent inhibition of
parathyroid cell function.
Cinacalcet has been effective in reducing serum calcium levels in patients with intractable
primary hyperparathyroidism and inoperable parathyroid carcinoma.
 Denosumab : RANKL antagonist- decreases bone resorption
 Hypoparathyroidism

 Defined as PTH level <10 pg/ml (10-65 pg/ml)

 Permanent hypoparathyroidism would be defined as requirement of therapeutic Vitamin D

and/or calcium replacement at 6 months or fasting albumin corrected serum calcium below
8 mg/dl
 Hereditary hypoparathyroidism
 A. Aplasia/hypoplasia of parathyroid glands
b. HDR syndrome

 Hypoparathyroidism
 Sensorineural Deafness
 Renal anomaly
 GATA 3 gene located on chromosome 10p14
c. Autoimmune hypoparathyroidism

 Parathyroid gland antibodies

 Autoimmune polyglandular disease type 1
Autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy

D. Autosomal dominant Hypoparathyroidism

Surgical hypoparathyroidism
 Pseudohypoparathyroidism

 Peripheral resistance to parathyroid hormone rather than a deficiency

 Hypocalcemia, hyperphosphatemia, raised serum PTH

 3 types:
 1a
 1b: renal resistance to PTH
 2: low calcium and high phosphate
Pseudohypoparathyroidism type 1A
Short 4th and 5th metacarpals
Round face
Short stature
Calcium deposits under the skin
Developmental delay
Dental hypoplasia
Soft tissue calcification/ossification
 Pseudopseudohypoparathyroidism

 Similar to pseudohypoparathyroidism 1a phenotypically

 Normal PTH levels, no peripheral resistance to PTH
 Serum calcium, serum phosphate levels normal
Treatment for hypoparathyroidism

 High calcium and low phosphorus diet

 Calcium carbonate daily
 Activated vitamin D analogues. Alfacalcidiol 1-3 mcg/d
 Vitamin D supplementation 25000-100000 IU/d to patients treated with activated vitamin
D analogues
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