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81 Cards in this Set

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MCC of tertiary hyperparathyroidism (least common type)

Post renal transplant (PTH raised long after calcium levels corrected)

White pulp of spleen undergoes necrosis due to

Beta hemolytic streptococcus

Pipe Stem hepatic fibrosis

Schistosomiasis

This leads to congestive splenomegaly

Splenic infarcts

White and bland

Hassall corpuscles

Thymic medullary epithelial cells

Thymic hyperplasia due to

Proliferation of B CELLS

MCC of thymic follicular hyperplasia

Myasthenia gravis

Thymomas

Tumors of thymic epithelial cells

Non invasive thymomas

Medullary type epithelial cells


Less thymocytes

Invasive thymomas

Cortical type epithelial cells


More thymocytes

MC type of thymic carcinoma

SCC

Second MC-lymphoepithelioma type (EBV causal)

MC variety of thymomas associated with Autoimmune dz

Cortical type

PARATHYROID GLANDS

pathology of parathyroid glands

PTH released by

Chief cells of PTH gland

Function of PTH

Increases ionized calcium in serum

-increases bone osteoclast action


-increases small bowel calcium and phosphate absorption (via Vit D )


-increases renal calcium reabsorption ( distal tubule )


-deceased renal phophate reabsorption ( proximal tubule)

MCC of primary hyperparathyroidism

Solitary Parathyroid adenomas

MCC of secondary hyperparathyroidism (compensatory raised PTH secretion)

Chronic renal failure

MCC of tertiary hyperparathyroidism (least common type)

Post renal transplant (PTH raised long after calcium levels corrected)

Causes of Hypercalcemia with raised PTH

1° hyperparathyroidism


2° hyperparathyroidism


3° hyperparathyroidism


Familial hypocalciuric hypercalcemia

Causes of Hypercalcemia with decreased PTH

Malignancy


Sarcoidosis


Vit D intoxication


Thiazides


Immobilization

Clinical feature of hypercalcemia

MCC of hypercalcemic crisis (Ca>15mg/dl)

CA breast

Excess calcium ingestion causes

Milk alkali syndrome (as antacids of calcium carbonate/milk)


Hypercalcemia


Alkalosis


Renal failure

Chronic form of DZ-BURNETT SYNDROME


Pancreatitis leads to

Hypo calcemia

Calcium levels?

Case of 1° hyperparathyroidism

Lab findings in primary hyperparathyroidism

Increased PTH


Increased Ca2+


Decreased PO43-


Increased urinary cAMP


Increased serum alkaline phosphatase


Lab findings in 2° hyperparathyroidism-



Decreased PO4 excretion


Increased serum PO4


Decreased serum Ca


Increased PTH (renal osteodystrophy)


Increased SALP


Radiological features of 1° hyperparathyroidism

Clinical features of primary hyperparathyroidism

-Bones (osteoporosis)


-Renal Stones (calcium oxalate)


-Abdominal Groans (constipation, acute pancreatitis, pud)


-Psychiatric Moans (depression, seizures)


-polyuria


-brown tumors (osteitis fibrosa cystica)-hallmark of severe hyperparathyroidism-von recklinghausen dz of bone


-proximal myopathy


-gout/pseudo gout


-nephrocalcinosis

T/T of 1° hyperparathyroidism-sx removal of affected gland

Vitamin intoxication leading to hypercalcemia

Vit A>D

Initial management of Hypercalcemia

MC presentation of 1° hyperparathyroidism in developing countries

Bones


Stones


Groans


Moans

MC presentation of 1° hyperparathyroidism in developed countries

Asymptomatic hypercalcemia

Are steroids useful in 1° hyperparathyroidism induced Hypercalcemia?

NO



Rugger jersey spine

Normal calcium levels

9-11 gm%

Signs in hypocalcemia

Trousseau sign (carpopedal spasm)


Chvostek sign (tapping facial nerve)


Circumoral tingling, numbness


Latent tetany


Prolonged QT interval

Hypocalcemia with hypophosphatemia

Vit D dependent rickets type 1

Hypocalcemia with hyperphophatemia

Hypoparathyroidism causes-


Autoimmune (MC)


Sx excision


DiGeorge syndrome

Pseudo pseudo hypoparathyroidism

Normal calcium


Normal phosphate

Pseudo hypoparathyroidism-


-end organ PTH resistance (PTH raised with decreased Ca)


-Autosomal dominant form (short ht+short IV, V digits)


Management of hypocalcemia in hypoparathyroidism

1. Calcitriol supplements


2. Oral calcium intake


3. Thiazides (to increase reabsorption)

Case of 2° hyperparathyroidism

Ca, PO4, serum ALP, PTH in OSTEOPOROSIS

All normal

Ca, PO4, serum ALP, PTH in PAGETS DZ

Ca, PO4, PTH-normal


SALP-highly increased


Ca, PO4, serum ALP, PTH in CRF

Ca-decreased


Rest all-increased

Ca, PO4, serum ALP, PTH in RICKETS

Ca-decreased


PO4-decreased


SALP-increased


PTH-increased

Ca, PO4, serum ALP, PTH in HYPERPARATHYROIDISM

Ca-increased


PO4-decreased


SALP-increased


PTH-increased

Case of hypercalcemic crisis

Stepwise management of Hypercalcemic crisis

1. IV FLUIDS (200-500ml/h NS for a UO of >100ml/h)


2. LOOP DIURETICS (furosemide to increase excretion)


3. Bisphophonates (to decrease calcium release from bones)

Calcium levels directly related to

Magnesium levels

Another ion)

MCC of asymptomatic Hypercalcemia in adults

1° hyperparathyroidism

MCC of symptomatic Hypercalcemia in adults

Malignancy

MC mech by which osteolytic tumors cause Hypercalcemia

PTHrP

ADRENAL MEDULLA

Pathology of adrenal medulla

Cells in adrenal medulla

Neural crest derived chromaffin cells

TX of chromaffin cells

Phaeochromocytoma

Also called as the great masquerader due to variable presentation

Triad of phaeochromocytoma (MC symptoms of phaeochromocytoma)

Headache-MC symptom


Profuse sweating


Palpitations/tachycardia

MC sign of phaeochromocytoma

Episodic hypertension (or sustained)

Other clinical features of phaeochromocytoma

Anxiety


Panic attacks


Wt loss


Polyuria


Polydipsia


Orthostatic hypotension


Erythrocytosis


Hyperglycemia


Hypercalcemia (due to PTHrP)


DCMP


Constipation

MC catecholamine from phaeochromocytoma

Norepinephrine

MC catecholamine from extra adrenal phaeochromocytoma

Norepinephrine

MC catecholamine from adrenal phaeochromocytoma

<3cm-epinephrine


>3cm-norepinephrine



(mc size-5cm)

MC catecholamine from malignant phaeochromocytoma

Norepinephrine

MC catecholamine from phaeochromocytoma in MEN syndromes

Epinephrine

Cellular pattern characteristic of phaeochromocytoma

Zelballen pattern


Salt and pepper chromatin



(also of paragangliomas)

Rule of tens

10% Familial


10% NOT associated with episodic HTN

Phaeochromocytoma with distant metastasis is called

Malignant phaeochromocytoma



(cellular atypia, mitotic figures, invasion of vessels and adjacent structures)

To distinguish malignant from benign phaeochromocytoma, best parameter?

Presence of metastasis

Associated syndromes with phaeochromocytoma

NF 1 (Cafe au lait spots, option nerve glioma)


MEN 2A (Medullary carcinoma of thyroid, PTH hyperplasia)


MEN 2B (Marfanoid habitus, medullary carcinoma of thyroid, ganglioneuromas)


VHL syndrome (RCC, pancreatic endocrine neoplasms)


Paraganglioma syndrome

NEVER do this diagnostic test in phaeochromocytoma

FNAC

It is vascular

Best investigation for phaeochromocytoma

24-h urinary fractionated metanephrines



Other investigations-


Increased serum metanephrines


Increased 24h urinary VMA

Gold standard for locating tumors in phaeochromocytoma

Fluorodopa-PET

DOC for HTN in phaeochromocytoma

Phenoxybenzamine


(irreversible alpha blocker)



(may be followed by beta blockers-propanolol)


(prazosin/phentolamine for parpxysms)



Other drugs-


Nitroprusside


Metyrosine

Drug NEVER GIVEN ALONE in phaeochromocytoma

Beta blockers

Chemotherapy protocol for phaeochromocytoma

TOC for residual phaeochromocytoma/malignant phaeochromocytoma/metastatic phaeochromocytoma

MIBG (nuclear med therapy)

MC site for extra adrenal phaeochromocytoma

Abdomen (organ of Zuckerkandl)



(More risk of malignancy in extra adrenal phaeochromocytomas)

Treatment of phaeochromocytoma

Surgical excision preceded by phenoxybenzamine pre op (to prevent hypertensive crisis)

MC mutations in Familial Paraganglioma

SDH (succinate dehydrogenase mutations)

Why the name 'chromaffin' for phaeochromocytoma?

Turns brown on incubation with potassium dichromate solution (d/T oxidation of stored catecholamines)

IHC Markers of phaeochromocytoma

Chromogranin


Synaptophysin

Special name for phaeochromocytomas in extra adrenal sites

Paragangliomas