ENDOCRINE DISORDERS

DR.SRINATH.CHANDRAMANI

Asst.Prof & ICU in-charge

K.J.Somaiya Medical College,

Mumbai

INTRODUCTION The practice of endocrinology is intimately linked to understanding :

a. hormone secretion,

b. hormone action, and

c. principles of feedback control systems.

Most disorders of the endocrine system are amenable to effective treatment, once the correct diagnosis is determined.

Endocrine deficiency disorders are treated with physiologic hormone replacement; hormone excess conditions, usually due to benign glandular adenomas, are managed by removing tumors surgically or by reducing hormone levels medically.

Hormone classes (1) amino acid derivatives such as dopamine, catecholamines, and thyroid hormone;

(2) small neuropeptides such as GnRH, TRH, somatostatin, and vasopressin;

(3) large proteins such as insulin, LH and PTH

(4) steroid hormones such as cortisol and estrogen & (5) vitamin derivatives such as retinoids (vitamin A and vitamin D.

Endocrine Concepts As a rule, amino acid derivatives and

peptide hormones interact with cell-surface membrane receptors.

Steroids, thyroid hormones, vitamin D, and retinoids are lipid-soluble and interact with intracellular nuclear receptors.

Endocrine Concepts

The physiologic functions of hormones can be divided into three general areas:

(1) growth and differentiation,

(2) maintenance of homeostasis, and

(3) reproduction.

Classification of Endocrine Disorders

Pituitary disorders

Thyroid disorders

Pancreatic disorders

Adrenal disorders

Bone disease

Pituitary disorders

Deficiency Hypopituitarism

Excess Gigantism/ Acromegaly

Hypopituitarism

Maybe due to lesion in Hypothalamus or pituitary gland.

Congenital Kallmanns syndrome

Acquired - Infections, Tumours, Injury

Radiotherapy, Hypoxic.

Kallmanns syndrome

KAL 1 gene defect anosmin

Codes for migration of progenitor cells.

Midline defects, Anosmia.

Characterised by Hypogonadotrophic hypogonadism (GnRH deficiency)

Multi-type inheritance

Acquired Hypopituitarism GH is 1st to be affected < LH< ACTH < TSH.

Stimulation test or individual hormone assays are diagnostic

Treatment indications

Hyperpituitarism Acromegaly/Gigantism their difference

Diagnosis : Clinical heel pad thickness

Lab : Insulin stress assay / GH assay

Treatment

HyperProlactinemia

Most common disturbance of pituitary

Causes : Physiology Drugs Prolactinomas

HyperProlactinemia Clinical features

Prolactin levels

TRH stimulation test

differentiate PRL tumour vs other causes.

Medical therapy

Indications for surgery

Misc Pituitary disorders Pituitary hyperplasia increased trophic hormones,

correct deficiency if any.

Empty sella syndrome

Craniopharyngioma 2nd decade,

Midline tumour, suprasellar calcification,

Manifests mainly as headaache as

pressure effects.

Diabetes Insipidus Inadequate Vasopressin/ ADH

Hypotonic Polyuria

Neurogenic vs Nephrogenic

Role of Desmopressin

Clorpropamide increases ADH secretion.

Role of Thiazides

Response to Vasopressin

SIADH Sustained ADH release

Hyponatremia with Concentrated urine

Oligo-anuria

Diagnosis

Treatment Demeclocycline to induce Nephrogenic DI

Water restriction.

Thyroid diseases

Hypothyroidism

Primary (disease in thyroid)

Secondary (Pituitary disease)

Hyperthyroidism

Graves disease (most common)

Hypothyroidism

Primary vs Secondary

Congenital vs Acquired

Detection

Treatment

Prognosis

Hyperthyroidism

Graves disease LATS

Jod-Basedow effect

De-Quervans thyroiditis

Hashimotos thyroiditis

Struma ovarii

Endogenous

Hyperthyroidism

Clinical features

Diagnosis

Role of Isotope scan

Treatment

Prognosis

Parathyroid Disorders

Chief cells secrete PTH

Parafollicular C cells secrete Calcitonin

Both are counter-regulatory

Pararthormone related peptide

Part of MEN1 and MEN 2.

Pancreatic disorders

Diabetes Mellitus

Glucagonoma

Insulinoma

Diabetes Mellitus

Type 1

MODY

Type 2

Others

Diabetes Mellitus

Diagnosis

Treatment

Complications

Special types : GDM

GDM Screen 24 weeks.

F > 105 mg/dl

1 Hr > 190 mg/dl

2 r > 165 mg/dl

Insulin is drug of choice.

Adrenal disorders Physiology :

ZONA GFR Aldosterone, Glucocorticoid, Sex hormones

Excess Hyperaldosteronism, Cushings

Deficiency Addisons

Hyperaldosteronism

Primary Conns syndrome

Secondary renin dependant causes

Hypokalemia

Diastolic hypertension

Metabolic alkalosis

Cushings syndrome

Central pituitary tumour (cushings disease)

Adrenal hyperplasia, tumours.

Ectopic secretion Medullary Ca thyroid

Addisons disease

Primary vs Secondary

Clinical features

Complications

Diagnosis

Treatment

Sex Hormone defects Ambiguous Genetalia

Congenital Adrenal Hyperplasia Iatrogenic virilisation

True Hermaphrodite

Precocious Puberty

Central / true idiopathic, tumours Ovarian / Pseudo Mccune albright

Testicular Feminising syndrome

Testicular Feminising syndrome

Also called Androgen Insensitivity syndrome

Defect in androgen receptor.

Hence testosterone is high

But female phenotype develops.

Genotype is XY.

Pheochromocytoma Rare, Benign Enterochrommafin cells

Commonest site is adrenal medulla (90%)

It is called a 10% tumour as :

10% bilateral

10% malignant

10% extra-adrenal

10% Familial

10% multiple

10% occur in children.

Pheochromocytoma No correlation between size and

catecholamine levels

Most secrete both Epinephrine & NE.

NorEpinephrine is dominantly secreted

Epinephrine dominant in MEN

Diagnosis : metanephrine, VMA levels.

CT/ Isotope imaging

Syndromes associated

MEN

Neurofibroma 1

Von-Hippel Landau syndrome

Acromegaly

Multiple Endocrine Neoplasia MEN 1 MEN 2 A MEN 2B

HYPERPARATHYROISM MEDULLARY CA OF THYROID

Medullary Ca of thyroid

HYPERPITUITARISM PTH ADENOMA Neurofibromas

ZES Bilateral adrenal hyperplasia

Thickened corneal nerves

Pheochromocytoma Pheochromocytoma Pheochromocytoma

HYPERADRENALISM Marfanoid Habitus