Fundamentals of Pathology
Husain A. Sattar
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Presents a s lower extremity cyanosis i n infants, often a t birth 2. Associated with Turner syndrome C. Adult form is not associated with a PDA; coactation lies after (distal to) the aortic arch. 1 . Presents as hypertension i n the upper extremities and hypotension with weak 1. pulses in the lower extremities; classically discovered in adulthood 2 . Collateral circulation develops across the intercostal arteries; engorged arteries cause 'notching' of ribs on x-ray (Fig. 8 . 1 1 B). 3.
AGENESIS A. Absent kidney formation; may be unilateral or bilateral B. Unilateral agenesis leads to hypertrophy of the existing kidney; hyperfiltration increases risk of renal failure later in life. C. Bilateral agenesis leads to oligohydramnios with lung hypoplasia, flat face with low set ears, and developmental defects of the extremities (Potter sequence, Fig. 1 2.2); incompatible with life III. DYSPLASTIC KIDNEY A. Non inherited, congenital malformation of the renal parenchyma characterized by
base of tongue mass HYPERTHYRO I D I S M I. BASIC PRINCIPLES A. Increased level of circulating thyroid hormone 1 . Increases basal metabolic rate (due t o increased synthesis of Na+ -K+ ATPase) 2. Increases sympathetic nervous system activity (due to increased expression of � 1 -adrenergic receptors) B. Clinical features include 1 . Weight loss despite increased appetite 2. Heat intolerance and sweating 3. Tachycardia with increased cardiac output 4. Arrhythmia (e.g., atrial fib rillation),
exacerbates hypotension, often leading to death. C . Chronic insufficiency (Addison disease) is due to progressive destruction of the adrenal glands. 1 . Common causes include autoimmune destruction (most common cause in 1. the West), TB (most common cause in the developing world), and metastatic carcinoma (e.g., arising from lung). 2 . Clinical features include hypotension, hyponatremia, hypovolemia, hyperkalemia, weakness, hyperpigmentation (increased ACTH by-products stimulate melanocytic
frontal and temporal cortex; spares the parietal and occipital lobes B. Characterized by round aggregates of tau protein (Pick bodies) in neurons of the cortex C. Behavioral and language symptoms arise early; eventually progresses to dementia V. PARKINSON DISEASE A. Degenerative loss of dopaminergic neurons in the substantia nigra of the basal ganglia (Fig. 17. 13A,B) l . Nigro striatal pathway of basal ganglia uses dopamine to initiate movement. B. Common disorder related to aging; seen in 2% of