{"id":37033,"date":"2026-06-12T07:08:55","date_gmt":"2026-06-12T01:38:55","guid":{"rendered":"https:\/\/atsixty.com\/?p=37033"},"modified":"2026-06-12T07:09:38","modified_gmt":"2026-06-12T01:39:38","slug":"hereditary-vascular-structural-nephrology","status":"publish","type":"post","link":"https:\/\/atsixty.com\/index.php\/morning-rounds\/hereditary-vascular-structural-nephrology\/","title":{"rendered":"Hereditary, Vascular &amp; Structural Nephrology"},"content":{"rendered":"\n\n\n<meta charset=\"UTF-8\">\n<meta name=\"viewport\" content=\"width=device-width, initial-scale=1.0\">\n<title>Morning Rounds \u00b7 Hereditary, Vascular &amp; Structural Nephrology<\/title>\n<link href=\"https:\/\/fonts.googleapis.com\/css2?family=Playfair+Display:ital,wght@0,400;0,600;0,700;1,400;1,600&#038;family=Source+Serif+4:ital,wght@0,300;0,400;0,600;1,400&#038;display=swap\" rel=\"stylesheet\">\n<style>\n#nep07 *,#nep07 *::before,#nep07 *::after{box-sizing:border-box;margin:0;padding:0}\n#nep07{\n  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font-size=\"9.5\" font-family=\"Georgia,serif\" font-weight=\"bold\">ADPKD &mdash; Extrarenal Manifestations<\/text>\n      <!-- Central kidney label -->\n      <ellipse cx=\"260\" cy=\"92\" rx=\"48\" ry=\"28\" fill=\"#2A5470\" opacity=\"0.15\"\/>\n      <text x=\"260\" y=\"88\" text-anchor=\"middle\" fill=\"#1C3D52\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">PKD1 \/ PKD2<\/text>\n      <text x=\"260\" y=\"100\" text-anchor=\"middle\" fill=\"#2A5470\" font-size=\"7.5\" font-family=\"Georgia,serif\">Polycystin defect<\/text>\n      <!-- Berry aneurysm -->\n      <rect x=\"10\" y=\"14\" width=\"140\" height=\"38\" rx=\"4\" fill=\"#fdf0f0\"\/>\n      <text x=\"80\" y=\"27\" text-anchor=\"middle\" fill=\"#B83232\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">Intracranial Berry Aneurysms<\/text>\n      <text x=\"80\" y=\"39\" text-anchor=\"middle\" fill=\"#7a1010\" font-size=\"7\" font-family=\"Georgia,serif\">5\u201310% of patients<\/text>\n      <text x=\"80\" y=\"50\" text-anchor=\"middle\" fill=\"#7a1010\" font-size=\"7\" font-family=\"Georgia,serif\">Most feared complication \u2014 SAH<\/text>\n      <line x1=\"150\" y1=\"38\" x2=\"214\" y2=\"78\" stroke=\"#B83232\" stroke-width=\"1.2\" stroke-dasharray=\"3,2\"\/>\n      <!-- Liver cysts -->\n      <rect x=\"370\" y=\"14\" width=\"140\" height=\"38\" rx=\"4\" fill=\"#eaf6ef\"\/>\n      <text x=\"440\" y=\"27\" text-anchor=\"middle\" fill=\"#2D6B47\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">Hepatic Cysts<\/text>\n      <text x=\"440\" y=\"39\" text-anchor=\"middle\" fill=\"#1a4a28\" font-size=\"7\" font-family=\"Georgia,serif\">Commonest extrarenal feature<\/text>\n      <text x=\"440\" y=\"50\" text-anchor=\"middle\" fill=\"#1a4a28\" font-size=\"7\" font-family=\"Georgia,serif\">Usually asymptomatic<\/text>\n      <line x1=\"370\" y1=\"38\" x2=\"306\" y2=\"78\" stroke=\"#2D6B47\" stroke-width=\"1.2\" stroke-dasharray=\"3,2\"\/>\n      <!-- Mitral valve prolapse -->\n      <rect x=\"10\" y=\"120\" width=\"140\" height=\"38\" rx=\"4\" fill=\"#fff5e0\"\/>\n      <text x=\"80\" y=\"133\" text-anchor=\"middle\" fill=\"#7a5000\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">Mitral Valve Prolapse<\/text>\n      <text x=\"80\" y=\"145\" text-anchor=\"middle\" fill=\"#5a3800\" font-size=\"7\" font-family=\"Georgia,serif\">~25% of patients<\/text>\n      <text x=\"80\" y=\"156\" text-anchor=\"middle\" fill=\"#5a3800\" font-size=\"7\" font-family=\"Georgia,serif\">Mid-systolic click<\/text>\n      <line x1=\"150\" y1=\"132\" x2=\"214\" y2=\"108\" stroke=\"#7a5000\" stroke-width=\"1.2\" stroke-dasharray=\"3,2\"\/>\n      <!-- Pancreatic cysts -->\n      <rect x=\"190\" y=\"148\" width=\"140\" height=\"22\" rx=\"4\" fill=\"#e8f3f9\"\/>\n      <text x=\"260\" y=\"161\" text-anchor=\"middle\" fill=\"#1C3D52\" font-size=\"7.5\" font-family=\"Georgia,serif\">Pancreatic cysts &nbsp;|&nbsp; Seminal vesicle cysts<\/text>\n      <!-- Aortic root -->\n      <rect x=\"370\" y=\"120\" width=\"140\" height=\"38\" rx=\"4\" fill=\"#f4eef8\"\/>\n      <text x=\"440\" y=\"133\" text-anchor=\"middle\" fill=\"#5a1a7a\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">Aortic Root Dilatation<\/text>\n      <text x=\"440\" y=\"145\" text-anchor=\"middle\" fill=\"#3a0a5a\" font-size=\"7\" font-family=\"Georgia,serif\">Aortic regurgitation<\/text>\n      <text x=\"440\" y=\"156\" text-anchor=\"middle\" fill=\"#3a0a5a\" font-size=\"7\" font-family=\"Georgia,serif\">Aortic dissection (rare)<\/text>\n      <line x1=\"370\" y1=\"132\" x2=\"306\" y2=\"108\" stroke=\"#5a1a7a\" stroke-width=\"1.2\" stroke-dasharray=\"3,2\"\/>\n    <\/svg>\n    <figcaption>\n      ADPKD is a systemic disease of polycystin dysfunction, not merely a renal condition. Intracranial berry aneurysms are the most clinically feared \u2014 screening MRA is recommended in those with a family history of SAH or high-risk occupations. Hepatic cysts are the commonest extrarenal finding but rarely cause portal hypertension.\n    <\/figcaption>\n  <\/figure>\n<\/div>\n\n<!-- HUS vs TTP comparison for Q5 debrief -->\n<div id=\"nep07-img5\" style=\"display:none\">\n  <figure class=\"mr-img-wrap\">\n    <svg viewBox=\"0 0 520 148\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" style=\"width:100%;max-width:520px;display:block;margin:0 auto\">\n      <rect x=\"0\" y=\"0\" width=\"520\" height=\"148\" rx=\"8\" fill=\"#f0f6fa\"\/>\n      <text x=\"260\" y=\"15\" text-anchor=\"middle\" fill=\"#1A2C38\" font-size=\"9.5\" font-family=\"Georgia,serif\" font-weight=\"bold\">Thrombotic Microangiopathy &mdash; HUS vs TTP<\/text>\n      <!-- Headers -->\n      <rect x=\"10\"  y=\"22\" width=\"120\" height=\"18\" rx=\"2\" fill=\"#2A5470\"\/>\n      <rect x=\"135\" y=\"22\" width=\"180\" height=\"18\" rx=\"2\" fill=\"#2A5470\"\/>\n      <rect x=\"320\" y=\"22\" width=\"190\" height=\"18\" rx=\"2\" fill=\"#1C3D52\"\/>\n      <text x=\"70\"  y=\"34\" text-anchor=\"middle\" fill=\"#F0F7FC\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">Feature<\/text>\n      <text x=\"225\" y=\"34\" text-anchor=\"middle\" fill=\"#F0F7FC\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">HUS (STEC-associated)<\/text>\n      <text x=\"415\" y=\"34\" text-anchor=\"middle\" fill=\"#F0F7FC\" font-size=\"8\" font-family=\"Georgia,serif\" font-weight=\"bold\">TTP<\/text>\n      <!-- Rows -->\n      <rect x=\"10\"  y=\"42\" width=\"120\" height=\"18\" rx=\"2\" fill=\"#d4e8f4\"\/>\n      <rect x=\"135\" y=\"42\" width=\"180\" height=\"18\" rx=\"2\" fill=\"#e8f3f9\"\/>\n      <rect x=\"320\" y=\"42\" width=\"190\" height=\"18\" rx=\"2\" fill=\"#f4eef8\"\/>\n      <text x=\"70\"  y=\"54\" text-anchor=\"middle\" fill=\"#1C3D52\" font-size=\"7.5\" font-family=\"Georgia,serif\">Mechanism<\/text>\n      <text x=\"225\" y=\"54\" text-anchor=\"middle\" fill=\"#143a4e\" font-size=\"7.5\" font-family=\"Georgia,serif\">Shiga toxin &rarr; endothelial injury<\/text>\n      <text x=\"415\" y=\"54\" text-anchor=\"middle\" fill=\"#3a0a5a\" font-size=\"7.5\" font-family=\"Georgia,serif\">ADAMTS13 deficiency &rarr; vWF multimers<\/text>\n\n      <rect x=\"10\"  y=\"62\" width=\"120\" height=\"18\" rx=\"2\" fill=\"#c4dff0\"\/>\n      <rect x=\"135\" y=\"62\" width=\"180\" height=\"18\" rx=\"2\" fill=\"#e8f3f9\"\/>\n      <rect x=\"320\" y=\"62\" width=\"190\" height=\"18\" rx=\"2\" fill=\"#f0eaf8\"\/>\n      <text x=\"70\"  y=\"74\" text-anchor=\"middle\" fill=\"#1C3D52\" font-size=\"7.5\" font-family=\"Georgia,serif\">Trigger<\/text>\n      <text x=\"225\" y=\"74\" text-anchor=\"middle\" fill=\"#143a4e\" font-size=\"7.5\" font-family=\"Georgia,serif\">E. coli O157:H7 (bloody diarrhoea)<\/text>\n      <text x=\"415\" y=\"74\" text-anchor=\"middle\" fill=\"#3a0a5a\" font-size=\"7.5\" font-family=\"Georgia,serif\">Autoantibody to ADAMTS13<\/text>\n\n      <rect x=\"10\"  y=\"82\" width=\"120\" height=\"18\" rx=\"2\" fill=\"#d4e8f4\"\/>\n      <rect x=\"135\" y=\"82\" width=\"180\" height=\"18\" rx=\"2\" fill=\"#fdf0f0\"\/>\n      <rect x=\"320\" y=\"82\" width=\"190\" height=\"18\" rx=\"2\" fill=\"#fdf0f0\"\/>\n      <text x=\"70\"  y=\"94\" text-anchor=\"middle\" fill=\"#1C3D52\" font-size=\"7.5\" font-family=\"Georgia,serif\">Dominant organ<\/text>\n      <text x=\"225\" y=\"94\" text-anchor=\"middle\" fill=\"#B83232\" font-size=\"7.5\" font-family=\"Georgia,serif\" font-weight=\"bold\">Kidney (AKI predominant)<\/text>\n      <text x=\"415\" y=\"94\" text-anchor=\"middle\" fill=\"#B83232\" font-size=\"7.5\" font-family=\"Georgia,serif\" font-weight=\"bold\">Brain (neuro symptoms predominant)<\/text>\n\n      <rect x=\"10\"  y=\"102\" width=\"120\" height=\"18\" rx=\"2\" fill=\"#c4dff0\"\/>\n      <rect x=\"135\" y=\"102\" width=\"180\" height=\"18\" rx=\"2\" fill=\"#e8f3f9\"\/>\n      <rect x=\"320\" y=\"102\" width=\"190\" height=\"18\" rx=\"2\" fill=\"#eaf6ef\"\/>\n      <text x=\"70\"  y=\"114\" text-anchor=\"middle\" fill=\"#1C3D52\" font-size=\"7.5\" font-family=\"Georgia,serif\">Treatment<\/text>\n      <text x=\"225\" y=\"114\" text-anchor=\"middle\" fill=\"#143a4e\" font-size=\"7.5\" font-family=\"Georgia,serif\">Supportive; avoid antibiotics<\/text>\n      <text x=\"415\" y=\"114\" text-anchor=\"middle\" fill=\"#2D6B47\" font-size=\"7.5\" font-family=\"Georgia,serif\" font-weight=\"bold\">Plasma exchange (URGENT)<\/text>\n\n      <rect x=\"10\"  y=\"122\" width=\"120\" height=\"18\" rx=\"2\" fill=\"#d4e8f4\"\/>\n      <rect x=\"135\" y=\"122\" width=\"180\" height=\"18\" rx=\"2\" fill=\"#e8f3f9\"\/>\n      <rect x=\"320\" y=\"122\" width=\"190\" height=\"18\" rx=\"2\" fill=\"#e8f3f9\"\/>\n      <text x=\"70\"  y=\"134\" text-anchor=\"middle\" fill=\"#1C3D52\" font-size=\"7.5\" font-family=\"Georgia,serif\">Age \/ context<\/text>\n      <text x=\"225\" y=\"134\" text-anchor=\"middle\" fill=\"#143a4e\" font-size=\"7.5\" font-family=\"Georgia,serif\">Children; food-borne outbreak<\/text>\n      <text x=\"415\" y=\"134\" text-anchor=\"middle\" fill=\"#143a4e\" font-size=\"7.5\" font-family=\"Georgia,serif\">Young women; idiopathic or drug<\/text>\n\n      <text x=\"260\" y=\"145\" text-anchor=\"middle\" fill=\"#7A9BAD\" font-size=\"7\" font-family=\"Georgia,serif\" font-style=\"italic\">Both: MAHA (schistocytes) + thrombocytopenia + negative Coombs<\/text>\n    <\/svg>\n    <figcaption>\n      The pentad of TTP (MAHA, thrombocytopenia, renal impairment, fever, neurological symptoms) is rarely complete \u2014 empirical plasma exchange should not wait for all five. ADAMTS13 activity &lt;10% confirms TTP. In STEC-HUS, antibiotics are avoided as they trigger toxin release.\n    <\/figcaption>\n  <\/figure>\n<\/div>\n\n<div id=\"nep07\">\n  <div class=\"mr-header\">\n    <div class=\"mr-eyebrow\">Morning Rounds \u00b7 Nephrology Series \u00b7 Round 07<\/div>\n    <div class=\"mr-title\">Hereditary, Vascular<br><em>&amp; Structural Nephrology<\/em><\/div>\n    <div class=\"mr-subtitle\">Five cases &middot; Read carefully &middot; Trust your instinct<\/div>\n    <div class=\"mr-chips\">\n      <span class=\"mr-chip\">5 Cases<\/span>\n      <span class=\"mr-chip\">+4 \/ &minus;1 scoring<\/span>\n      <span class=\"mr-chip\">Options reshuffled<\/span>\n    <\/div>\n  <\/div>\n\n  <div class=\"mr-sentinel\" id=\"nep07-sentinel\"><\/div>\n  <div class=\"mr-progress\" id=\"nep07-progress\">\n    <div class=\"mr-prog-inner\"><div class=\"mr-pips\" id=\"nep07-pips\"><\/div><\/div>\n  <\/div>\n\n  <div class=\"mr-body\">\n    <div id=\"nep07-cases\"><\/div>\n    <div class=\"mr-submit-wrap\">\n      <button class=\"mr-btn\" id=\"nep07-submit\">Submit for Debrief<\/button>\n    <\/div>\n    <div class=\"mr-score\" id=\"nep07-score\">\n      <div class=\"mr-score-in\">\n        <div class=\"mr-score-ey\">Round Complete<\/div>\n        <div class=\"mr-ring\" id=\"nep07-ring\">\n          <div class=\"mr-ring-in\">\n            <span class=\"mr-ring-pct\" id=\"nep07-pct\">0%<\/span>\n            <span class=\"mr-ring-sub\">net<\/span>\n          <\/div>\n        <\/div>\n        <div class=\"mr-score-title\">Your Debrief<\/div>\n        <div class=\"mr-score-net\" id=\"nep07-net\"><\/div>\n        <div class=\"mr-verdict\" id=\"nep07-verdict\"><\/div>\n        <div class=\"mr-bands\">\n          <span class=\"mr-band mr-band-c\" id=\"nep07-ct-c\"><\/span>\n          <span class=\"mr-band mr-band-w\" id=\"nep07-ct-w\"><\/span>\n          <span class=\"mr-band mr-band-s\" id=\"nep07-ct-s\"><\/span>\n        <\/div>\n        <button class=\"mr-retry\" id=\"nep07-retry\">&#8635; New Round<\/button>\n      <\/div>\n    <\/div>\n  <\/div>\n<\/div>\n\n<script>\n(function(){\n  'use strict';\n  var NS='nep07',TOTAL=5,MAX=20,LTRS=['A','B','C','D'];\n\n  var QS=[\n    {\n      id:1,\n      tag:'Structural Nephrology &mdash; ADPKD: Diagnosis',\n      stem:'A <strong>34-year-old man<\/strong> presents with bilateral flank discomfort and haematuria. His father died of a <strong>subarachnoid haemorrhage<\/strong> at age 52 and was known to have \"large kidneys.\" Ultrasound shows <strong>bilaterally enlarged kidneys with multiple cysts<\/strong> in both kidneys and liver. BP is 158\/96 mmHg. Creatinine is 1.3 mg\/dL. Genetic testing shows a <strong>PKD1 mutation<\/strong>. Which statement about ADPKD is most accurate?',\n      correct:'PKD1 mutations (chromosome 16) account for ~85% of cases and carry a worse prognosis than PKD2; ESRD occurs a decade earlier',\n      opts:[\n        'PKD1 mutations (chromosome 16) account for ~85% of cases and carry a worse prognosis than PKD2; ESRD occurs a decade earlier',\n        'ADPKD is autosomal recessive; both parents must be carriers for the disease to manifest',\n        'PKD2 mutations are more common and more severe; PKD1 is a benign variant',\n        'Liver cysts in ADPKD indicate hepatic polycystic disease \u2014 a separate, unrelated condition'\n      ],\n      exp:'<strong>ADPKD<\/strong> is the commonest inherited kidney disease (1 in 400\u20131000), inherited in an <strong>autosomal dominant<\/strong> pattern \u2014 a single mutant allele suffices. <strong>PKD1<\/strong> (polycystin-1, chromosome 16p) accounts for <strong>~85%<\/strong> of cases and causes ESRD at a median age of <strong>54 years<\/strong>. <strong>PKD2<\/strong> (polycystin-2, chromosome 4q) accounts for ~15% and has a milder course (ESRD median age ~74). Both polycystins are mechanosensory proteins in primary cilia; their dysfunction leads to dysregulated tubular epithelial proliferation and fluid secretion, forming progressively enlarging cysts. Diagnosis by ultrasound uses the <strong>Ravine criteria<\/strong> (age-dependent cyst counts). <strong>Total kidney volume (TKV)<\/strong> on MRI is the best predictor of progression. Hypertension is the earliest and commonest manifestation \u2014 present in 60% by age 30, driven by intrarenal RAAS activation. Treatment: <strong>tolvaptan<\/strong> (V2-receptor antagonist) slows TKV growth in rapidly progressive disease (TEMPO trial).',\n      imgId:null\n    },\n    {\n      id:2,\n      tag:'Structural Nephrology &mdash; ADPKD: Extrarenal Features',\n      stem:'The same patient (Q1) asks about risks beyond his kidneys. His maternal aunt died of a <strong>brain bleed<\/strong> at 48. He is a commercial airline pilot. Which extrarenal complication carries the <em>highest immediate mortality risk<\/em>, and what is the appropriate screening recommendation for this patient specifically?',\n      correct:'Intracranial berry aneurysm rupture (SAH); MRA brain screening is indicated given positive family history of SAH and high-risk occupation',\n      opts:[\n        'Intracranial berry aneurysm rupture (SAH); MRA brain screening is indicated given positive family history of SAH and high-risk occupation',\n        'Hepatic cyst infection; annual ultrasound of liver is mandatory in all ADPKD patients',\n        'Mitral valve prolapse with severe regurgitation; annual echocardiography should be performed',\n        'Pancreatic cysts with malignant transformation; MRCP every 2 years from diagnosis'\n      ],\n      exp:'ADPKD is a systemic disease. <strong>Intracranial (berry) aneurysms<\/strong> occur in <strong>5\u201310%<\/strong> of ADPKD patients (vs ~1% in the general population) and are the most feared complication \u2014 rupture causes subarachnoid haemorrhage with 30\u201350% mortality. <strong>Screening with MRA brain<\/strong> is <em>not<\/em> recommended universally in ADPKD but is specifically indicated when: (1) <strong>family history of SAH or intracranial aneurysm<\/strong>, (2) <strong>high-risk occupation<\/strong> (pilots, surgeons, drivers), (3) prior aneurysm, or (4) patient anxiety after counselling. This patient has <em>both<\/em> risk factors \u2014 unambiguous indication. Other extrarenal features: <strong>hepatic cysts<\/strong> (commonest, ~80% by age 60, usually asymptomatic), <strong>mitral valve prolapse<\/strong> (~25%), aortic root dilatation, pancreatic and seminal vesicle cysts. Hepatic cysts rarely cause portal hypertension. Hepatic cyst infection presents with fever and right upper quadrant pain \u2014 treated with fluoroquinolones (lipid-soluble, penetrate cysts well).',\n      imgId:'nep07-img2'\n    },\n    {\n      id:3,\n      tag:'Vascular Nephrology &mdash; Renal Artery Stenosis',\n      stem:'A <strong>28-year-old woman<\/strong> with no prior medical history presents with difficult-to-control hypertension (BP 172\/106 mmHg on two agents). On auscultation there is a <strong>high-pitched systolic-diastolic bruit<\/strong> in the right flank. Renal duplex Doppler shows elevated peak systolic velocity in the right renal artery. MRA reveals a <strong>beaded appearance<\/strong> of the mid and distal right renal artery. Serum creatinine is normal. What is the diagnosis and treatment of choice?',\n      correct:'Fibromuscular dysplasia (FMD) of the renal artery; percutaneous transluminal angioplasty (PTA) without stenting',\n      opts:[\n        'Fibromuscular dysplasia (FMD) of the renal artery; percutaneous transluminal angioplasty (PTA) without stenting',\n        'Atherosclerotic renal artery stenosis; stenting is the first-line intervention',\n        'Takayasu arteritis; high-dose corticosteroids are the treatment of choice',\n        'Essential hypertension with renal bruit; no further investigation required'\n      ],\n      exp:'<strong>Fibromuscular dysplasia (FMD)<\/strong> is a non-inflammatory, non-atherosclerotic arteriopathy affecting medium-sized arteries, predominantly in <strong>young women<\/strong>. The renal arteries (right &gt; left) and carotid\/vertebral arteries are most commonly involved. The pathognomonic <strong>\"string of beads\"<\/strong> appearance on angiography\/MRA reflects alternating areas of stenosis and dilation in the media of the vessel wall. Unlike atherosclerotic RAS (ostial, proximal, in older patients with cardiovascular risk factors), FMD affects the mid and distal renal artery. <strong>Treatment: percutaneous transluminal angioplasty (PTA) alone<\/strong> \u2014 stenting is generally avoided as technical success rates are high with balloon angioplasty and long-term patency is excellent. Stenting is reserved for failed PTA or dissection. Atherosclerotic RAS: the ASTRAL and CORAL trials showed that renal artery stenting offered no benefit over optimal medical therapy in most patients \u2014 a landmark finding that changed practice. ACE inhibitors\/ARBs are contraindicated in bilateral RAS or RAS in a solitary kidney (risk of AKI from loss of efferent arteriolar tone).',\n      imgId:null\n    },\n    {\n      id:4,\n      tag:'Vascular Nephrology &mdash; Malignant Hypertension',\n      stem:'A <strong>45-year-old man<\/strong> presents with severe headache, visual blurring, and BP <strong>228\/136 mmHg<\/strong>. Fundoscopy shows <strong>bilateral papilloedema and flame haemorrhages<\/strong>. Serum creatinine is 4.2 mg\/dL (baseline unknown). Urine microscopy shows <strong>RBC casts and heavy proteinuria<\/strong>. Blood film shows <strong>schistocytes<\/strong>. What is the diagnosis and the key principle governing antihypertensive management?',\n      correct:'Malignant hypertension with hypertensive nephrosclerosis and microangiopathic haemolytic anaemia; reduce MAP by no more than 20\u201325% in the first hour \u2014 avoid precipitous falls',\n      opts:[\n        'Malignant hypertension with hypertensive nephrosclerosis and microangiopathic haemolytic anaemia; reduce MAP by no more than 25% in the first hour \u2014 avoid precipitous falls',\n        'Hypertensive urgency without end-organ damage; oral antihypertensives over 24\u201348 hours are appropriate',\n        'TTP presenting with hypertension; plasma exchange is the priority over BP control',\n        'Glomerulonephritis with secondary hypertension; treat the GN first and BP will normalise'\n      ],\n      exp:'<strong>Malignant (accelerated) hypertension<\/strong> is defined by severely elevated BP with <em>evidence of acute end-organ damage<\/em> \u2014 here papilloedema (grade IV hypertensive retinopathy), hypertensive nephrosclerosis (RBC casts, proteinuria, AKI), and <strong>microangiopathic haemolytic anaemia (MAHA)<\/strong> from fibrin deposition in arterioles shearing RBCs (schistocytes). Pathophysiology: fibrinoid necrosis of small vessels &#x2192; ischaemia &#x2192; further RAAS activation &#x2192; vicious cycle. <strong>Management principle<\/strong>: this is a hypertensive <em>emergency<\/em> requiring IV antihypertensives (labetalol, nicardipine, or sodium nitroprusside). The critical rule: <strong>reduce MAP by no more than 20\u201325% in the first hour<\/strong>, then to 160\/100 over the next 2\u20136 hours. <em>Precipitous<\/em> BP reduction causes cerebral, coronary, and renal hypoperfusion (autoregulation has shifted rightward). Target normalisation over 24\u201348 hours. The renal function often <em>worsens transiently<\/em> before improving as BP is controlled \u2014 do not be alarmed by an initial creatinine rise. The MAHA resolves with BP control, distinguishing it from TTP.',\n      imgId:null\n    },\n    {\n      id:5,\n      tag:'Vascular Nephrology &mdash; HUS &amp; TTP',\n      stem:'A <strong>6-year-old girl<\/strong> develops <strong>bloody diarrhoea<\/strong> after a school picnic. Five days later she becomes oliguric, pale, and irritable. Investigations: Hb <strong>6.8 g\/dL<\/strong>, platelets <strong>38 &times; 10&#x2079;\/L<\/strong>, creatinine <strong>5.2 mg\/dL<\/strong>, blood film shows <strong>schistocytes<\/strong>, direct Coombs test <strong>negative<\/strong>. Stool culture grows <em>E. coli<\/em> O157:H7. Neurological examination is normal. What is the diagnosis and the most important treatment <em>to avoid<\/em>?',\n      correct:'Haemolytic uraemic syndrome (STEC-HUS); antibiotics must be avoided \u2014 they lyse bacteria and trigger massive Shiga toxin release',\n      opts:[\n        'Haemolytic uraemic syndrome (STEC-HUS); antibiotics must be avoided \u2014 they lyse bacteria and trigger massive Shiga toxin release',\n        'Thrombotic thrombocytopenic purpura (TTP); urgent plasma exchange is the treatment of choice',\n        'Immune thrombocytopenic purpura with AKI; IV immunoglobulin and platelet transfusion are indicated',\n        'Haemolytic uraemic syndrome; plasma exchange should be started immediately as in TTP'\n      ],\n      exp:'The triad of <strong>MAHA + thrombocytopenia + AKI<\/strong> following a prodrome of bloody diarrhoea in a child confirms <strong>STEC-HUS<\/strong> (Shiga toxin-producing <em>E. coli<\/em>, classically O157:H7). Mechanism: Shiga toxin is absorbed from the gut, binds to Gb3 receptors on glomerular endothelial cells &#x2192; endothelial injury &#x2192; microvascular thrombosis &#x2192; AKI + MAHA. The negative Coombs test excludes autoimmune haemolysis. <strong>Critical avoidance: antibiotics<\/strong> \u2014 bactericidal antibiotics cause bacterial lysis with <em>massive Shiga toxin dump<\/em>, worsening the clinical course. Antiperistaltic agents (loperamide) are similarly contraindicated. <strong>Treatment is supportive<\/strong>: careful fluid management, dialysis if needed, red cell transfusion for symptomatic anaemia (platelets only if active bleeding \u2014 transfused platelets fuel more microvascular thrombosis). <strong>Plasma exchange is NOT indicated in STEC-HUS<\/strong> \u2014 it is the cornerstone of <em>TTP<\/em> (where ADAMTS13 deficiency allows ultra-large vWF multimers to drive platelet aggregation). The absence of neurological symptoms and the diarrhoeal prodrome clinch STEC-HUS over TTP here.',\n      imgId:'nep07-img5'\n    }\n  ];\n\n  var answers={},answered=0,shuffled={},done=false;\n  function byId(id){return document.getElementById(id);}\n  function gid(s){return byId(NS+'-'+s);}\n  function shuffleArr(arr){var a=arr.slice(),i,j,tmp;for(i=a.length-1;i>0;i--){j=Math.floor(Math.random()*(i+1));tmp=a[i];a[i]=a[j];a[j]=tmp;}return a;}\n  function countVal(val){var k,n=0;for(k in answers){if(answers.hasOwnProperty(k)&&answers[k]===val)n++;}return n;}\n  function buildPips(){\n    var cont=gid('pips'),i,q,wLine,wPip,line,pip;cont.innerHTML='';\n    for(i=0;i<QS.length;i++){\n      q=QS[i];\n      if(i>0){wLine=document.createElement('div');wLine.className='mr-pip-wrap';line=document.createElement('div');line.className='mr-pip-line';line.id=NS+'-pl'+q.id;wLine.appendChild(line);cont.appendChild(wLine);}\n      wPip=document.createElement('div');wPip.className='mr-pip-wrap';pip=document.createElement('div');pip.className='mr-pip';pip.id=NS+'-pip'+q.id;pip.textContent=String(q.id);wPip.appendChild(pip);cont.appendChild(wPip);\n    }\n  }\n  function build(){\n    var cont,i,q,opts,card,top,numDiv,meta,tag,stem,rule,optsDiv,expDiv,lbl,txt,imgDiv,imgSrc,j,optEl,ltrSpan,txtSpan;\n    cont=gid('cases');cont.innerHTML='';answers={};answered=0;shuffled={};done=false;gid('score').style.display='none';buildPips();\n    for(i=0;i<QS.length;i++){\n      q=QS[i];opts=shuffleArr(q.opts);shuffled[q.id]=opts;\n      card=document.createElement('div');card.className='mr-case';\n      top=document.createElement('div');top.className='mr-case-top';\n      numDiv=document.createElement('div');numDiv.className='mr-num';numDiv.textContent=q.id<10?'0'+q.id:String(q.id);\n      meta=document.createElement('div');meta.className='mr-meta';\n      tag=document.createElement('div');tag.className='mr-tag';tag.innerHTML=q.tag;\n      stem=document.createElement('div');stem.className='mr-stem';stem.innerHTML=q.stem;\n      meta.appendChild(tag);meta.appendChild(stem);top.appendChild(numDiv);top.appendChild(meta);card.appendChild(top);\n      rule=document.createElement('div');rule.className='mr-rule';card.appendChild(rule);\n      optsDiv=document.createElement('div');optsDiv.className='mr-opts';\n      for(j=0;j<opts.length;j++){\n        optEl=document.createElement('div');optEl.className='mr-opt';optEl.id=NS+'-o'+q.id+'-'+j;optEl.setAttribute('role','button');optEl.setAttribute('tabindex','0');\n        ltrSpan=document.createElement('span');ltrSpan.className='mr-ltr';ltrSpan.textContent=LTRS[j];\n        txtSpan=document.createElement('span');txtSpan.className='mr-opt-text';txtSpan.innerHTML=opts[j];\n        optEl.appendChild(ltrSpan);optEl.appendChild(txtSpan);optsDiv.appendChild(optEl);\n        (function(qid,oi){optEl.addEventListener('click',function(){pick(qid,oi);});}(q.id,j));\n      }\n      card.appendChild(optsDiv);\n      expDiv=document.createElement('div');expDiv.className='mr-exp';expDiv.id=NS+'-exp'+q.id;\n      lbl=document.createElement('div');lbl.className='mr-exp-lbl';lbl.textContent='Debrief';\n      txt=document.createElement('div');txt.className='mr-exp-text';txt.innerHTML=q.exp;\n      expDiv.appendChild(lbl);expDiv.appendChild(txt);\n      if(q.imgId){imgSrc=byId(q.imgId);if(imgSrc){imgDiv=document.createElement('div');imgDiv.innerHTML=imgSrc.innerHTML;expDiv.appendChild(imgDiv);}}\n      card.appendChild(expDiv);cont.appendChild(card);\n    }\n  }\n  function pick(qid,oi){\n    var q,opts,i,el,correct;\n    if(answers[qid]!==undefined||done)return;\n    q=null;for(i=0;i<QS.length;i++){if(QS[i].id===qid){q=QS[i];break;}}if(!q)return;\n    opts=shuffled[qid];correct=(opts[oi]===q.correct);answers[qid]=correct?'c':'w';answered++;\n    for(i=0;i<opts.length;i++){\n      el=byId(NS+'-o'+qid+'-'+i);\n      if(opts[i]===q.correct){el.className='mr-opt correct locked';}\n      else if(i===oi){el.className='mr-opt wrong locked';}\n      else{el.className='mr-opt dimmed locked';}\n    }\n    byId(NS+'-exp'+qid).style.display='block';\n    byId(NS+'-pip'+qid).className='mr-pip '+(correct?'correct':'wrong');\n    if(qid>1){var pl=gid('pl'+qid);if(pl)pl.className='mr-pip-line done';}\n  }\n  function showScore(){\n    var c,w,s,net,pct,disp,verdicts,vi,sc;\n    if(done)return;done=true;\n    c=countVal('c');w=countVal('w');s=TOTAL-answered;\n    net=(c*4)-w;pct=Math.max(0,Math.round((net\/MAX)*100));disp=Math.min(100,Math.max(0,pct));\n    gid('ring').style.background='conic-gradient(#2A5470 '+disp+'%, #D8E6EE 0%)';\n    gid('pct').textContent=pct+'%';gid('net').textContent='Net Score: '+net+' \/ '+MAX;\n    verdicts=[[5,'Perfect series closer. 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Structural Nephrology ADPKD &mdash; Extrarenal Manifestations PKD1 \/ PKD2 Polycystin defect Intracranial Berry Aneurysms 5\u201310% of patients Most feared complication \u2014 SAH Hepatic Cysts Commonest extrarenal feature Usually asymptomatic Mitral Valve Prolapse ~25% of patients Mid-systolic click Pancreatic cysts &nbsp;|&nbsp; Seminal vesicle cysts Aortic Root Dilatation Aortic regurgitation Aortic&hellip;&nbsp;<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"","neve_meta_content_width":0,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","footnotes":""},"categories":[74,80],"tags":[],"class_list":["post-37033","post","type-post","status-publish","format-standard","hentry","category-morning-rounds","category-nephrology"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.8 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Hereditary, Vascular &amp; 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