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Endocrine - Adrenal Gland (GFR, CAH) (*Adrenal Glands GFR zonas (dropped…

- - - - Medulla = *Epi and NE
        
        Catecholamine Synthesis
        
        Tyrosine --> DOPA --> dopamine --> NE --> Epi
        
        Tyrosine --> DOPA = rate limiting step
        
        TYRANTS are DOPE and they make PerMNT (permanent) Epi
        
        Catecholamine Synthesis example
        
        Case Presentation:
        
        Notes:
        
        note PNMT = phenyl .. - N methyl transferase = last step to make Epi in the Tyrosine --> DOPA line
        
        note that ACTH also leaks from the cortex into the medulla to increase the PNMT and Epi synthesis
        
        Catecholamine Release
        
        from the adrenal medulla --> cat in the bed
        
        stimulated by ACh release for Sympathetic NS
        
        Notes:
        
        solution = adrenal medulla stimmed by ACH from SNS
        
        note that the adrenal medulla makes catechols
        --> 80% mostly = epi, 20% norepi
        
        Clinical Case
      - *GFR hormones
        
        "the deeper you go the sweeter it gets"
        
        *Cortisol = Zona Fascicula
        -
        
        *Metyrapone test for cortisol levels and HP-Adrenal axis
        
        blocks conversion of 11-hydrocortisone to cortisol in the adrenal gland
        --> increases blood serum 11-hydrocortisol
        --> increases urine 17-
        
        stopping cortisol stops the negative inhibition of cortisol on the hypothal and pit
        --> gives hyper ACTH release
        
        *Adrenal crisis
        
        can't raise the cortisol levels int he body to respond to stress
        
        the most common cause of this is chronic glucocorticoid use
        
        nlong term prednisone causes atrophy of the CRH releasing cells of the hypothal
        --> then that atrophies the ACTH cells of the anterior pit
        --> then the FR of adrenal GFR atrophy and you lose the ability to make cortisol in response to stress
        
        this is called adrenal crisis
        
        usually this can happen in surgeries
        
        Clinical Cases
        
        1 more item...
        
        *Aldosterone = Zona Glomerulus
        -
        
        *Addisons Disease
        
        "Addisons = Aldosterone Deficient"
        
        *Conns Syndrome Disease
        
        "CONNs = TONS of aldosterone"
        --> hyperaldosterone
        
        Tx of Conns syndrome
        
        either surgery to remove primary tumors
        
        tx = spironolactone, epleronone
        --> ONE = aldosterone blockers at the late DCT and CD
        --> principle PT cells and alpha - IC cells with H+ pumps
        
        P/C of Conns Syndrome
        
        recall that aldosterone acts ont he DCT and CD
        
        increases excretion of K+ and H+
        
        hypo H+ --> gives metabolic alkylosis
        
        hypo K+ --> gives muscle pains
        
        1 more item...
        
        *DHEA Androgens = Zona Reticularis
        -
    - - Pheochromocytoma
        
        adrenal medulla benign tumor
        
        chromaffin cells
        --> chromocytoma
        
        secretes atecholamines (Epi and NE)
        
        Notes:
        
        Pheochromocytoma is a benign tumor of the adrenal medulla
        
        it is a catecholamine producing tumour
        --> tachycardia
        --> paroxysmal hypertensive episodes
        
        Case Presentation:
        
        Paraganglioma
        
        Differential to Pheochromocytoma
        
        .
        
        Clinical Case
        
        Notes:
        
        note that the above, it doesn't matter that it is a paraganglioma or a pheochromocytoma
        --> both produce excessive NE and Epi.
        --> same clinical symptoms with high sympathetic NS: H/A, palpitations, acute HTN
        
        NE and Epi. are both metabolised into normetanephrine and metanephrine
        --> META nephrine - think of meta as being downstream of NE and Epi.
        --> these would be raised in both the blood and in the urine
      - *Cushing Disease
        
        Endogenous
        --> either primary or
        --> secondary = central
        
        exogenous = steroid use
        
        *dexamethasone test to diagnose Cushing Disease
        -
        
        *DIRTY USMAL
        *CUSHINGS disease
        
        1 more item...
        
        BAE is FAT with cortisol
        
        CUSHING 3 places you can get it
        think of CUSHIN for the PUSHINin JAIL
        look out in ALL 3 directions for SOAP DROPPPERS
        --> 3 separate gangs in the showers
        --> the DOMES and the SIDES
        SYNDROME in the SIDE
        DISEASE in the DOME
        
        1 more item...
- - - - ⇩ 17 alpha A-H
        
        ⇩ 17 alpha A-H: ("17 = age you want to sexually develop and enter the Androstenedione pathway")
        
        key is bigh BP and low adrostenedione
        --> low adrostenedione = AMENORRHEA
        
        decreased LH in 17 alpha hydroxylase
        
        you have less estrogen in 17 AH deficiency
        --> no negative feedback to hypothal
        --> keep producing high FSH and LH
        
        Clinical Cases
        
        Clinical Case
        
        Clinical Case
        
        Notes:
        
        note that
        
        Clinical Case
        
        Clinical Case
        
        Notes:
        
        note that
      - ⇩ 21 A-H:
        
        MOST COMMON of the Congenital CAH disorders
        
        ("21 age limit for entry to GF = GET FUCKED pathway")
      - ⇩ 11 Beta A-H
        
        ("11 = partial entry to 21 age limit for entry to GF = GET FUCKED pathway")
    - - CAH disorder Presentations
        
        CAH are a cluster of disorders that ALL have 2 things in common
        --> LOW cortisol
        --> HIGH ACTH
        
        high ACTH acts on the melanocytes also and increases melatonin
        --> High ACTH gives high skin pigmentation