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1/2 Liddle Syndrome
| Question | Answer |
|---|---|
| HTN: __/__ sustained taken __. Pre-HTN: __/__ to __/__.HTN is the MC serious chronic disease in __. | 140/90, BILATERALLY, 120/80, 139/89, African Americans |
| Tumor of the adrenal medulla. | PHEOCHROMOCYTOMA |
| Pheochromocytoma: secretes excessive amts __: __, __, __. ↑Urinary excretion of __ (__). ↑Plasma __ w/ normal __. | CATECHOLAMINES, EPINEPHRINE, NE, DOPAMINE, 3-methoxy-4-hydroxymandelic acid (VMA), CATECHOLAMINES, T4 |
| Most common adrenal medullary tumor. | PHEOCHROMOCYTOMA |
| Pheochromocytoma is a tumor derived from the __ that arise from the __. It is associated with __ & __. | CHROMAFFIN CELLS, NEURAL CREST, MEN2 & 3, NEUROFIBROMATOSIS |
| Aldosterone: mineralocorticoid produced by the __, the outermost layer of the __. It's synthesized from __ b/c of low__, low __ ratio in plasma & by __. | ZONA GLOMERULOSA, ADRENAL CTX, CHOLESTEROL, BLOOD VOL, NA+/K+, ANGIOTENSIN II |
| Aldosterone forms when the __ of the __ release __ in response to __. Renin cleaves __ -> __. ACE (__) converts __ -> __. __ causes the adrenal gland to secrete __. | JUXTAGLOMERULAR CELLS, KIDNEY, RENIN, LOW BV, ANGIOTENSINOGEN, ANGIOTENSIN I, ANGIOTENSIN CONVERTING ENZYME, ANG I, ANG II, ANG II, ALDOSTERONE |
| Cortisol: __ produced by the __, the __ of the adrenal cortex. Synthesized from __ in response to __. | GLUCOCORTICOID, ZONA FASCICULATA, MIDDLE LAYER, CHOLESTEROL, STRESS |
| During stress the __ sends __ to the __, which in turn sends __ to the __. This structure then produces __, which inhibits __ & __. | HYPOTHALAMUS, CRH, ANT PITUITARY, ACTH, ADRENAL CTX, CORTISOL, ANT PIT, HYPOTHAL |
| Adrenal Medulla comes from the __. Secretes __ (__ and __). | NEUROECTODERM, CATECHOLAMINES, EPINEPHRINE, NE |
| Adrenal Cortex comes from the __. Secretes __. | MESODERM, ADRENOCORTICOSTEROID HORMONES |
| What are the 3 zones of the adrenal cortex & what do they secrete? | Go Find Rex - Salt Sugar Sex = Glomerulosa, Fasiculata, Reticularis = Salt (mineralocorticoids), Sugar (glucocorticoids), Sex (androgens) |
| The basic building block for adrenocorticosteroid hormones is __. The 21-Carbon steroids are: __, __, __, __. | CHOLESTEROL, CHOLESTEROL, PROGESTERONE, GLUCOCORTICOIDS, MINERALOCORTICOIDS. |
| __ is the precursor for the others in the 21-C series. | PROGESTERONE. |
| 19-Carbon steroids: __ & precursors to __.A 21-C steroid -> 17α-hydroxylase -> 17,20-lyase -> __ & __. | ANDROGENS, ESTROGENS, DHEA, ANDROSTENEDIONE |
| 18-Carbon steroids: have __ activity. __ to estrogens occurs in __ & __. | ESTROGENIC, AROMATIZATION, OVARIES, PLACENTA |
| Each layer of the adrenal cortex secretes a different type of __ due to the presence or absence of __. | STEROID HORMONE, ENZYMES |
| Cholesterol -> __ catalyzed by __. Cholesterol is present in all layers of the __ & is stimulated by __. | PREGNENOLONE, CHOLESTEROL DESMOLASE, CORTEX, ACTH |
| Pregnenolone -> Corticosterone occurs in __ steps using these enzymes: __, __, __. Corticosterone -> Aldosterone occurs by __, which is activated by __. | 3, 3β-hydroxysteroid dehydrogenase, 21β-hydroxylase, & 11β-hydroxylase, ALDOSTERONE SYNTHASE, ANG II |
| __ (__) & __ also have some mineralocorticoid actions. ZG cannot produce glucocorticoids b/c it lacks __. | 11-Deoxycorticosterone (DOC), CORTICOSTERONE, 17α-hydroxylase |
| __ is the primary product of ZF, which has the same 3 enzymes as __. But ZF has __ so that it can produce cortisol. __ also has some glucocorticoid action. | CORTISOL, ZG, 17α-hydroxylase, CORTICOSTERONE |
| __ & __ are the major products of the ZR. They have weak __ activity. Can be converted into __ in the __. ZR has the enzyme __ which is required for __ synthesis from __. | DHEA, ANDROSTENEDIONE, ANDROGENIC, TESTOSTERONE, TESTES, 17,20-LYASE, ANDROGEN, CHOLESTEROL |
| Cortisol activates __ receptors just as well, but not in __ b/c cortisol is __ and __. | ALDOSTERONE, KIDNEY, CONVERTED, EXCRETED |
| 19-Carbon steroids: androgens & precursors to estrogens.A __ -> __ -> __ -> DHEA & androstenedione. | 21-C STEROID, 17α-HYDROXYLASE, 17,20-LYASE |
| Aldosterone circulates mostly bound to __. Mineralocorticoid receptors are located in __ of __ & __ in nephron. | ALBUMIN, PRINCIPAL CELLS, DISTAL TUBULE, COLLECTING DUCTS |
| 11-Deoxycorticosterone (DOC) is secreted at ~same rate as __. Mostly bound to __ (__). Equal mineralocorticoid activity as __.Less effect than __ b/c there is __ in serum. | ALDOSTERONE, CORTICOSTEROID-BINDING GLOBULIN (CBG), ALDOSTERONE, ALDOSTERONE, LESS FREE |
| Cortisol effect on renal Na+-K+ transport: Similar affinity for R as __. Much higher free levels, but doesn’t exert as much __. Converted to __ via __ in kidneys, which isn't present everywhere in body. Inhibition of this enzyme -> __. | ALDOSTERONE, MINERALOCORTICOID AXN, CORTISONE, 11β-HYDROXYSTEROID DEHYDROGENASE, MINERALOCORTICOID HTN |
| CRH-containing neurons are in the __ of the __. Upon stimulation CRH is released into __ blood & delivered to the __. - CRH binds Rs on __ of the __, which release __, stimulating synthesis of __ (ACTH precursor) & release of __. | PARAVENTRICULAR NUCLEI, HYPOTHALAMUS, HYPOTHALAMIC-HYPOPHYSIAL PORTAL, AP, CORTICOTROPHS, AP, cAMP, POMC, ACTH |
| ACTH from AP: ↑ steroid hormone synthesis in __ of AC by __ stimulation of __ & ↑ cholesterol -> __. - ACTH also up-regulates __, so sensitivity of AC to ACTH __. Chronically ↑ levels of ACTH cause __. | ALL ZONES, cAMP, CHOLESTEROL DESMOLASE, PREGNENOLONE, ITS OWN RECEPTOR, INCREASES, AC HYPERTROPHY |
| Negative feedback of cortisol: inhibits __ from __ & __ from __. __(__) suppression test: ↓dose __ normally suppresses __ secretion. In __ tumors, it does not – but ↑ dose does. Neither ↓ nor ↑ dose __ inhibits __ secretion in __ tumors. | CRH, HYPOTHALAMUS, ACTH, AP, DEXAMETHASONE (DM), DM, ACTH, ACTH-SECRETING, DM, CORTISOL, ADRENAL CORTICAL |
| Low Renin Essential Hypertension (LREH) accounts for __ of pts w/ essential HTN. Possibly d/t __. Tx may be best w/ __ & __. | ~20%, MINERALOCORTICOID EXCESS, CA2+ CHANNEL BLOCKERS, DIURETICS |
| High Renin Essential Hypertension (HREH) accounts for __ of pts w/ essential HTN. Plasma renin levels >normal range. MC in __ & __. | ~15%, AFRICAN AMERICAN, ELDERLY |
| Essential HTN d/t Abnormal Cardiovascular Devo, ex. abnormal __. Possible ↓devo of __. | AORTIC ELASTICITY, MICROVASCULAR NETWORK |
| 2 types of Renal HTN (Essential HTN). | RENOVASCULAR HTN, RENAL PARENCHYMAL DISEASE |
| Renovascular HTN: ↓ __ b/c __. Activates the __. | RENAL PERFUSION, RENAL ARTERY STENOSIS, RENIN-ANGIOTENSIN SYSTEM |
| Renal Parenchymal Disease: ↓ renal perfusion b/c __ & __ to __. Activates __. Generally TX = __ b/c of activated __. | INFLAMMATORY, FIBROTIC CHANGES, RENAL BV's, RENIN-ANGIOTENSIN SYSTEM, ACE INHIBITORS, R-A SYSTEM, |
| Primary Aldosteronism: HTN from aldosterone induced __. __ very common (↑ __ → ↓ __). ↑Aldosterone -> ↓__ levels (__). Tx w/ __. | NA+ RETENTION, HYPOKALEMIA, NA+, K+, RENIN, FEEDBACK INHIBITION, MINERALOCORTICOID RECEPTOR AGONISTS |
| Main type of HTN = __ = __% hypertensive pts. Onset usually between __ years. | PRIMARY/ESSENTIAL/IDIOPATHIC HTN, 90-95%, 25-55 |
| Primary HTN epidemiology: Prevalence increases __. More prevalent in __ than __. MC in __. After __, HTN more common in __. | WITH AGE, MIDDLE-AGED MALES, FEMALES, AFRICAN AMERICAN, 60Y, WOMEN THAN MEN |
| Cortisol oscillates w/ __ (__ period). C levels highest __ ( | |
| __) & lowest __ ( | |
| __). GC essential for __. | CIRCADIAN RHYTHM, 24 HR, B/4 WAKING, 8AM, IN THE EVENING, MIDNIGHT, STRESS RESPONSE |
| Cortisol increases __, & __ in muscle. It decreases __ so __ available to liver. It decreases __ & __ in adipose tissue. It increases __ providing more __ to the liver. | GLUCONEOGENESIS, PTN CATABOLISM, PTN SYNTH, AA’S, GLUCOSE UTILIZATION, INSULIN SENSITIVITY, LIPOLYSIS, GLYCEROL |
| Anti-inflammatory effects of cortisol: Induce __ synthesis; a __ inhibitor. Inhibit __ production & thus proliferation of __. Pharm: prevents __. Inhibit __ & __ release from __ & __. | LIPOCORTIN, PHOSPHOLIPASE A¬¬2, IL-2, T-LYMPHOCYTES, REJECTION OF TRANSPLANTED ORGANS, HISTAMINE, SEROTONIN, MAST CELLS, PLTS |
| Cortisol maintains vascular response to __. Upregulates __ on arterioles & ↑vasoconstrictor effect of __. So w/ ↑cortisol, arterial P __, w/ ↓ cortisol, arterial P __. Cortisol increases __ AND __! Bad for __! | CATECHOLAMINES, α1-RECEPTORS, NE, INCREASES, DECREASES, BP, BLOOD GLUCOSE, ATHEROSCLEROSIS |
| Thiazide diuretics: in the short term excrete more & H2O. In the long term plasma volume approaches normal, but peripheral resistance __. Also worry about __ w/ thiazides! | Na+, H2O, DECREASES, HYPOKALEMIA |
| ACE inhibitors: block ACE that converts __ (which is a __). Less aldosterone causes __ Na+ & H2O retention. Also inhibits __. | ANG I-ANG II, VASOCONSTRICTOR, DECREASED, VASOCONSTRICTION |
| Angiotensin receptor blockers (ARBs) block __ angiotensin II recepters causing __. Very selective – ARB SEs __ ACE SEs. | VASCULAR, VASODILATION, ARE LESS THAN |
| Ca2+ channel blocker (CCBs) relax arteriolar __ to dilate arterioles & decrease __. | SMOOTH MUSCLE, DILATE, PERIPHERAL VASCULAR RESISTANCE |
| Vasodilators relax __ smooth muscle & increase plasma __ (not good). | [RENIN] |
| B-blockers (__ agents) decrease __ & __ output by acting on in heart. May also decrease __ outflow & inhibit release of __. | ADRENERGIC, HR, CARDIAC OUTPUT, B1 ADRENORECEPTORS, SYMPATHETIC, RENIN |
| Alpha-blockers decrease __ & __ – relax __ & __ smooth muscle. | PERIPHERAL VASCULAR RESISTANCE, ARTERIAL BP, ARTERIAL, VENOUS |
| People who show a marked decrease in BP from decreased Na+ intake, or vice versa = Na+-sensitive (S-S). Several S-S genes ID’d. Usually mutation in ptn making up 1 of the __ or __ in kidney __. The most studied ones affect __ (like __). | ION CHANNELS, TRANSPORTERS, EPITHELIUM, SODIUM CHANNELS, LIDDLE’S |
| Channels are designed to keep most Na+ __, hormones dictate sm amt let __. They can be mutated into a __ position so that Na+ & H2O flow freely __. In a large % of cases channels are no longer responsive to __ so pts also classified as __. | OUT, IN, “FIXED-OPEN”, BACK IN, RENAL HORMONES, “LOW-RENIN HYPERTENSIVES” |
| Salt sensitives a much greater risk of major problems assoc/w HTN (__, stroke, etc.) Can be passed genetically – should be able to test for it using __. | HEART FAILURE, STROKE, SNP’S |
| Low renin essential HTN (LREH) is HTN caused by factors other than high renin secretion, such as: __, __, __, __. | MINERALOCORTICOID EXCESS, ABNORMAL CA2+ METABOLISM, INCREASED TISSUE RENIN-ANTIOTENSIN ACTIVITY, PROBLEMS W/ EPITHELIAL NA2+ CHANNELS |
| Mineralcorticoid excess (ex. __). Tx: __ (aldosterone receptor __). Our patient’s mineralcorticoid levels were normal to low. | ALDOSTERONE, EPLERONONE, ANTAGONIST |
| Abnormal calcium metabolism. Tx: __ & __. Our patient’s calcium levels were normal. | DIURETICS, CA2+ CHANNEL BLOCKERS |
| Increased tissue renin-angiotensin activity could be in __ like kidneys, brain, adrenal glands, etc. We ruled this out w/ plasma __, __, __, & urine __ tests. Would be responsive to __. | SPECIFIC TISSUES, RENIN, ALDOSTERONE, CORTISOL, ALDOSTERONE, ACE INHIBITORS |
| Problems with epithelial sodium channels (ex. Liddles syndrome). Positive family history of __ along with __. | JUVENILE HTN, HYPOKALEMIA |
| Physiological features of LREH: occupies __% of pts w/ essential htn. Features inc: __ sensitivity (__% pts w/ essential htn), __ responsiveness, & __. | AROUND 20%, SALT, AROUND 60%, DIURETIC, FAMILY HISTORY |
| Renin is an __ & it’s main fxn is to __. A decrease in BP is detected by __ in the __ of the kidney. This causes __ to convert to renin in the __ cells. Renin secretion by these cells cleaves __ to __. | ENZYME, MAINTAIN BP, MECHANORECEPTORS, AFFERENT ARTERIOLES, PRORENIN, JUXTAGLOMERULAR, ANGIOTENSINOGEN, ANG I |
| Angiotensinogen synthesized & secreted by the __ & stimulated by: __, __, __, __, __ & __. | LIVER, INFLAMMATION, INSULIN, ESTROGENS, GLUCOCORTICOIDS, THYROID HORMONE, ANG II |
| ACE (__) from the __ & __ converts __ to __. This causes the __ cells in the adrenal cortex to synthesize & secrete __. | ANGIOTENSIN CONVERTING ENZYME, KIDNEYS, LUNGS, ANG I, ANG II, ZG, ALDOSTERONE |
| Aldosterone increases __ reabsorption, which increases __ because __ follows __. This is slow-acting b/c aldosterone’s actions require __ & __ in the kidney. | NA2+, BLOOD VOLUME, H2O, NA2+, GENE TXN, PTN SYNTH |
| Angiotensin II vasoconstricts __, increases __ & __ reabsorption, & acts on the __ to increase __ & stimulate __ secretion. | ARTERIOLES, NA2+, HCO3-, HYPOTHALAMUS, THIRST, ADH |
| The Macula Densa is located next to __. When NaCl increases it releases __ to decrease __. When NaCl decreases it releases __ to increase __. | JUXTAGLOMERULAR CELLS, ADENOSINE, RENIN, PROSTAGLANDINS, RENIN |
| When intrarenal baroreceptors sense increased pressure in the afferent arterioles, they respond by __. When they sense increased pressure, they respond by __. | DECREASING RENIN RELEASE, INCREASING RENIN RELEASE |
| Adrenergic Receptor Pathway: NE is released from postganglionic __ nerves. This stimulates __ in the __ cells to __. | SYMPATHETIC, ß1 receptors, JG, INCREASE RENIN RELEASE |
| Short-loop feedback in regulation of Renin secretion: Ang II directly stimulates __ receptors on __ cells -> decreased __ release (this is an example of product inhibition). | ANGIOTENSIN, JG CELLS, RENIN |
| Long Loop Negative Feedback in regulation of Renin secretion: increased pressure in __ arterioles reduces __ reabsorption in __ of the kidney. | AFFERENT, NACL, PROXIMAL TUBULE |
| ENaCs are tetrameric ptns: __α, __β, & __γ subunits – ea coded for by diff genes. The subunits have cytoplasmic __ & __ termini. 2 __ domains (M1 & M2), & a large glycosylated __-rich __ domain. | 2, 1, 1, N, C, TRANSMEMBRANE, CYSTEINE, EXTRACELLULAR |
| The 2 main cell types in the late distal tubule & collecting ducts. ENaCs are located on the __ membrane of the __ cells. | PRINCIPAL CELLS, INTERCALATED CELLS, LUMINAL, PRINCIPAL |
| Principal cells are for: __ reabsorption & __ secretion | H2O, NA+, K+ |
| Intercalated cells are for: __ reabsorption & __ secretion | K+, H+ |
| ENaC allows Na+ to flow __ its [gradient] out the lumen of the __ & into __ to be __. [Gradient] is created by __ Na+-K+-ATPase that pumps Na+ __ the interstitium & K+ __ the interstitium. Along w/ the kidney, the ENaC is also found in the __ & the __. | DOWN, CORTICAL COLLECTING TUBULE, EPITHELIAL CELLS, REABSORBED, BASOLATERAL, INTO, INTO CELL FROM, LUNG, DISTAL COLON |
| The main source of ENaC regulation is thru the hormone __, which is secreted by the __ of the __ & delivered to the __ cells via blood across the __ membrane. | ALDOSTERONE, ZG, ADRENAL CTX, PRINCIPAL CELLS, BASOLATERAL |
| Turning off the ENaC channel: The cytoplasmic __of either the __ subunit contains a __ domain (PPxY). The PPxY motif binds __, a ubiquitin ligase protein that promotes internalization & degradation of ENaC. | C TERMINUS, β OR γ, PROLINE-RICH, NEDD-4 |
| 1 way ALD regs renal ENaC= _ expression in tissue. C-steroids=> _ channel insertion into _of renal tubule _. ALD binds _, which _where a hormone-R complex _ ENaC subunits. _membrane ENaCs means there's _open channels, so _ Na reabsorption. Occurs over _. | INCREASING, MORE, LUMINAL MEMBRANE, EPITHELIAL CELLS, CYTOSOLIC RECEPTORS (hormone receptors – not part of ENaC), TRANSLOCATE TO THE NUCLEUS, ENHANCES TXN OF, MORE, MORE, MORE, SEVERAL HOURS-DAYS |
| MOA of Insulin/Ald interaxn to _ channel activity: I binds its renal cell plasma-memb R activating _ pathway, while ald increases inactive _ levels. _ phosphorylates _, activating it. Active _ binds & phosphorylates _. | INCREASE, PIP3 – PDK1, SGK (another ptn), PDK1, SGK, SGK, NEDD-4, INTERNALIZATION, DEGRADATION, 1HR |
| Liddle’s is autosomal _ => 1o increase in _ reabsorption & _ excretion from ¬_ & _. Mutations of genes on chromosome _ encoding _ subunits affect the cytoplasmic _ -> elimination of __ amino acids. Frameshift mutation causes premature __ -> truncated _. | DOMINANT, NA+, K+, DCT, COLLECTING DUCT, 16, β & γ, C TERMINUS, 45-75, STOP CODON, C TERMINUS |
| Normally, the PPxY motif binds Nedd-4, a _ ptn, that promotes _ & _ of ENaC molec’s. W/o this => _ of ENaCs & _ of Na+ ions, causing: pseudo_, low renin (_), increased _ of ENaC, Na+ _, & K+ _. | UBIQUITIN-LIGASE, INTERNALIZATION & DEGRADATION, CONSTITUTIVE EXPRESSION, REABSORPTION, HYPERALDOSTERONISM, VOL-EXPANDED HTN, BASAL ACTIVITY, REABSORPTION, EXCRETION |
| Liddle’s Sx: ¬_, hypokalemic metabolic _, looks like hyperaldosteronism, but almost absent _ & _ secretion, _ urine K+, _ urine Na+, _ serum Na+. | HTN, ALKALOSIS, RENIN, ALDOSTERONE, INCREASED, DECREASED, INCREASED |
| Liddle’s Tx: _ supplements, _ or _; _-sparing diuretics that directly close _ channel. | K+, TRIAMTERENE, AMILORIDE, K+, NA+ |
| SE’s for K+-sparing diruetics: _, _, _, _. | HA, CRAMPING, DIARRHEA, VOMITING |
| Spironolactone: Synthetic _ – direct _ antagonist. Competitively binds _ R’s in Na-K exchange in _ & _. _ inhibition => _ Na+ absorption & _ K+ excretion. Also => _ Ca2+ excretion d/t direct effect on _. It is _ for Tx of Liddle’s. | STEROID, ALDOSTERONE, ALD, DCT, COLLECTING DUCTS, ALD, DECREASED, DECREASED, INCREASED, TUBULAR TRANSPORT, INEFFECTIVE |
| Amiloride blocks Na+-K+ ion channel in _ preventing Na+ _. D/t Na-K exchange in DCT & CDs, prevents K+ _ in DCT. _ urine prodxn b/c _ leaves w/ _. Works independent of _ presence. For HTN pts w/ _, usually combined w/ _ or _. To prevent hyperkalemia, _. | DCT, REABSORPTION, EXCRETION, INCREASES, H20, NA+, ALD, LOW K+, THIAZIDES, LOOP DIURETICS, INCREASE CA2+ REABSORPTION |
| Triamterene blocks_ Na/Kchannel=direct inhibit Na_,=> memb e- potential that blocks_. During minimal K+ loss, only slightly modifies_. Significantly lowers_ when K+ renal clearance is high(¬_). Works indep of_presence. Shorter½-life than_. Metabolized in_ | DCT, REABSORPTION, PASSIVE K+ SECRETION, DCT K+ HANDLING, K+ EXCRETION, ALD, AMILORIDE, LIVER |
| 3 most common bacterial causes of acute OM? Chronic OM? | Acute: S. pneumoniae, H. influenza, M. catarrhalis (Pneumonia, flu, catarrh), Chronic: S. aureus, P. aeruginosa |
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