Disorders of Adrenal Glands Dr Mohammadzadeh Embryology The
Disorders of Adrenal Glands Dr. Mohammadzadeh
Embryology �The adrenal glands are two endocrine organs in one : cortex and medulla �Cortex originates in fifth week of gestation from mesoderm �Medulla is ectodermal and originate from neural crest �Most extraadrenal neural tissues regresses
Anatomy �Retroperitoneal organs at the level of the eleventh rib �Measures 5 x 3 x 1 cm and weight 4 -5 gr �Cortex 80 -90% of gland have three zones : glomerulosa, fasciculata, reticularis �Medulla 10 -20 % of gland
Hyperaldosteronism (Conns syn. ) �Results from autonomous aldostrone secretion �Between the ages of 30 -50 years and 1% of cases of hypertension �Usually �Causes 70% 30% associated with hypokalemia : solitary adrenal adenoma idiopathic bilateral hyperplasia
Symptoms and Signs �Moderate to severe long-standing hypertension and difficult to control �Muscle weakness , polydipsia , polyuria , nocturia , headaches and fatigue
Lab study �In any hypertensive patients with spontaneous hypokalemia (k <3. 2) or hypokalemia (k<3) while on diuretic therapy despite k replacement �Elevated PAC & suppressive PRA �PAC : PRA ratio of 25 to 30: 1 �Sodium loading test
Radiologic study �CT-scan �MRI sensitivity 90% are less sensitive �Selective venous catheterization �Scintigraphy with NP-59
Treatment �Unilateral tumors : adrenalectomy �Carcinoma : adrenalectomy �Bilateral adrenal hyperplasia : medical therapy �Patients who more likely benefit from surgery : response to spironolactone shorter duration of hypertension minimal end organ damage �Patients who least likely benefit : older than 50 years multiple adrenal nodules
Adrenocortical cancer �Are rare : 2 per 1 million �Bimodal age distribution : in children and in adults in the forth and fifth decade
Symptoms & signs � 50% are non-functional � 30% are cortisol secretion � 20% androgen secretion � 10% estrogen secretion � 2% aldostrone secretion �Non-functioning tumors : enlarging abdominal mass and abdominal pain
Cushings syndrom �A complex of symptoms & signs resulting from hypersecretion of cortisol
Diagnostic tests �The aims are twofold : to confirm the presense of Cushing syn. to determine its ethiology
Treatment � Adrenal � Primary adenoma : unilateral adrenalectomy adrenal hyperplasia : bilateral adrenalectomy � Cushing disease : transsphenoidal excision of the pituitary adenoma stereotactic radiosurgery chemical adrenalectomy bilateral adrenalectomy � Ectopic ACTH : management of primary tumor medical adrenalectomy bilateral adrenalectomy
Pheochromocytoma �Incidence 1. 9% in biochemical screening �In fourth & fifth decades no gender predilection �Extra-adrenal tumors ; paraganglioma in : organ of Zuckerkandl , neck , mediastinum , abdomen and pelvis
10 % Tumors � 10% bilateral � 10% malignant � 10% in pediatric � 10% extra-adrenal � 10% familial
Familial Pheochromocytoma �With MEN 2 A & MEN 2 B �In von. Hippel-Lindau disease �In Neurofibromatosis type 1 �Carney syndrome
Symptoms & Signs �Classic triad : Headache , palpitation , diaphoresis �Anxiety , paresthesias , flushing , chest pain , abdominal pain , nausia , vomiting �MI , CVA �Most common clininical sign : hypertension in 0. 1 -0. 2 %
Biochemical studies �Urinary metanephrines �Urinary VMA �Fractionated urinary catecholamines �In borderline levels : provocative tests �Clonidine suppression test �Chromogranin A �Most reliable test : plasma metanephrine
Radiologic studies �CT-scan without contrast �MRI � I-MIBG scan
Treatment �Tratment of choice : adrenalectomy completely and minimal manipulation or rupture of capsule
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