{"id":8111,"date":"2021-11-15T22:59:47","date_gmt":"2021-11-15T20:59:47","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/nephrology\/cystic-renal-disease-renal-tubular-disease-hypo-and-hyperkalaemia\/"},"modified":"2022-01-18T16:46:24","modified_gmt":"2022-01-18T14:46:24","slug":"cystic-renal-disease-renal-tubular-disease-hypo-and-hyperkalaemia","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/nephrology\/cystic-renal-disease-renal-tubular-disease-hypo-and-hyperkalaemia\/","title":{"rendered":"Cystic renal disease, renal tubular disease, hypo- and hyperkalaemia"},"content":{"rendered":"<span class=\"block-heading\" id=\"header_1\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Cystic disease<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">A cyst is an epithelium-lined sac of fluid. They are commonly found incidentally in the kidney as solitary lesions.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Cystic kidney disease is a group of conditions in which the kidney develops numerous cysts.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">They can be acquired or congenital.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Acquired cysts<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<ul class=\"wp-block-list\"><li>Most common in patients with CKD, specifically those on dialysis for many years, probably due to hyperplasia of the residual functioning tissue.<\/li><li>Usually asymptomatic, but may cause bleeding and hematuria.<\/li><li>Increase the risk for RCC. Patients with multiple acquired cysts should be screened using US.<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Congenital cysts<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<p class=\"wp-block-paragraph\">There are many causes for congenital cystic renal disease. The most prominent ones are <strong>polycystic kidney disease, nephronophthisis, <\/strong>and<strong> medullary cystic kidney disease.<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Polycystic kidney disease<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">PKD is generally classified into two entities, they are they <strong>ADPKD<\/strong> and <strong>ARPKD<\/strong> (a third entity, sporadic PKD, also exists, but is extremely rare).<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>ADPKD is more common, less severe, manifesting later in life, caused by a defect in the PKD1\/2 gene. CKD and renal failure commonly appears in late adulthood.<\/li><li>ARPKD is more rare, more severe, caused by a defect in the PKHD1 gene. Renal failure is common in childhood.<\/li><li>The kidneys are enlarged; hepatic cysts are also common (with minimal impairment), intracerebral berry aneurysm (AD).<\/li><li>In intrauterine kidney failure (ARPKD) = Potter syndrome (oligohydromnios &#8211; lung hypoplasia, limb and face abnormalities)<\/li><li>Compared with acquired cysts, there&#8217;s no increased risk for RCC.<\/li><li>Diagnosis: imaging, genetic testing<\/li><li>Treatment: ACEi\/ARB, Tolvaptan (AHD2r inhibitor), dialysis, nephrectomy and kidney transplant<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Nephronophthisis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<p class=\"wp-block-paragraph\">An AR disorder that leads to the development of cysts in the medulla and corticomedullar border, most often due to NPHP1 gene mutations. Nephronophthisis leads to CKD and renal failure <strong>early in life<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Inflammation of the tubules = tubulointerstitial nephritis, polyuria and uremia<\/li><li>Liver fibrosis is also common<\/li><li>Diagnosis using imaging and genetic testing<\/li><li>Treatment: Supportive<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">MCKD<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<p class=\"wp-block-paragraph\">MCKD is similar to nephroptosis except it appears in adulthood, and with no liver fibrosis. Presents with gout.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Most often due to MUC1 gene mutations. Leads to CKD and renal failure <strong>in adulthood<\/strong>.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Renal tubular disease<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<p class=\"wp-block-paragraph\">A heterogeneous group of diseases associated with defective renal tubular transport, leading to fluid loss, and electrolyte and acid-base disbalance.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_7\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Renal tubular acidosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_7\">\n\n\n<ul class=\"wp-block-list\" id=\"block-c8595fde-30f1-4da1-9525-087a6bb35c1e\"><li>Caused by a defect in <strong>H+ secretion<\/strong> (type I), <strong>HCO3- reabsorption<\/strong> (type II), <strong>CA deficiency<\/strong> (type III), or <strong>hypoaldosteronism\/aldosterone resistance<\/strong> (type IV)<\/li><li>Can be inherited or acquired<\/li><li>NAGMA<\/li><li>Typically asymptomatic<\/li><li><strong>Diagnosed <\/strong>using blood chemistry, and measurement of serum aldosterone and renin levels (decreased in type IV).<\/li><\/ul>\n\n\n\n<figure class=\"wp-block-table\"><table class=\"pure-table\"><thead><tr><th>Characteristic<\/th><th>Type I (distal) <\/th><th>Type II (proximal) <\/th><th> Type III (mixed) <\/th><th>Typer IV (generalized\/hyperkalemic) <\/th><\/tr><\/thead><tbody><tr><td>Etiology<\/td><td>Idiopathic<br>Inherited<br>Autoimmune<br>Sickle-cell<br>Drugs<\/td><td>Idiopathic<br>Inherited<br>Fanconi syndrome<br>Drugs<br>Multiple myeloma<\/td><td>Inherited<\/td><td>Primary\/secondary hypoaldosteronism<br>Diabetic nephropathy<br>Chronic interstitial nephritis<\/td><\/tr><tr><td>Defect<\/td><td>H+ secretion <\/td><td>HCO3- reabsorption <\/td><td>CA deficiency<\/td><td>Hypoaldosteronism\/aldosterone resistance<\/td><\/tr><tr><td>Characteristics<\/td><td>Rare<br>Hypercalciuria<br>Nephrolithiasis<\/td><td>Very rare<\/td><td>Extremely rare<\/td><td>Common<br>Hyperkalemia<br>Hyperammonemia<\/td><\/tr><tr><td>Urinary pH<\/td><td>>5.5<\/td><td>>7.5<\/td><td>>5.5<\/td><td>&lt;5.5<\/td><\/tr><tr><td>Treatment<\/td><td>Sodium citrate\/bicarbonate<\/td><td>Potassium citrate<br>Na+ restriction<\/td><td>Sodium\/potassium citrate<\/td><td>Fludrocortisone<br>K+ restriction <\/td><\/tr><\/tbody><\/table><\/figure>\n\n\n<\/span><span class=\"block-heading\" id=\"header_8\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Other diseases<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_8\">\n\n\n<ul class=\"wp-block-list\"><li><strong>X-linked hypophosphatemic rickets<\/strong> (defective reabsorption of phosphate -&gt; Vit-D-resistant rickets)<\/li><li><strong>Bartter syndrome<\/strong> (defective reabsorption of Cl-)<\/li><li><strong>Hartnup Syndrome<\/strong> (amino-acid transporter defect in the kidney and GIT, leading to pallegra-like symptoms [niacin deficiency])<\/li><li><strong>Fanconi Syndrome<\/strong> (defective proximal tubule transporters, leading to RTA type II, glucosuria, proteinuria, phosphaturia, calciuria, polyuria and dehydration; skeletal patholigies)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_9\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Hypokalemia<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_9\">\n\n\n<p class=\"wp-block-paragraph\">Normal K+ levels: 3.6-5.2mmol\/L.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Hypokalemia can be classified based on the cause:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Loss in the GIT (low urine K+)<ul><li>Vomiting, diarrhea, enema<\/li><\/ul><\/li><li>Loss in the kidneys (high urine K+)<ul><li>RTA type 1 and 2<\/li><li>Diuretics<\/li><li>Hyperaldosteronism<\/li><li>Hypomagnesemia<\/li><\/ul><\/li><li>Transcellular shift (low urine K+)<ul><li>Insulin<\/li><li>Beta-2 mimmetics<\/li><li>Alkalosis<\/li><\/ul><\/li><li>Inadequate intake (low urine K+)<\/li><li>Pseudohypokalemia (normal K+)<ul><li>Late sample analysis<\/li><li>Leukocytosis<\/li><\/ul><\/li><\/ul>\n\n\n\n<p class=\"wp-block-paragraph\">Presents with arrhythmias, decreased deep tendon reflexes, muscle spasms.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">ECG changes: T-wave flattening and inversion, ST-depression, prominent U wave<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Treatment: treat the underlying cause, supplement with oral KCl and magnesium if needed, if severe: IV KCl.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_10\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Hyperkalemia<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_10\">\n\n\n<p class=\"wp-block-paragraph\">Can be due to <strong>increased total potassium<\/strong>, <strong>redistribution<\/strong> of intracellular calcium, and <strong>pseudohyperkalemia<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Increased total K+<ul><li>Renal failure<\/li><li>Hypoaldosteronism<\/li><li>ACEi<\/li><li>K+-sparing diuretics<\/li><li>Blood transfusion<\/li><\/ul><\/li><li>Redistribution<ul><li>RTA (type 4)<\/li><li>Rhabdomyolysis<\/li><li>GI bleeding<\/li><li>Insulin deficiency<\/li><li>Rapid BB administration<\/li><\/ul><\/li><li>Pseudohyperkalemia<ul><li>Laboratory\/blood sampling error<\/li><li>Thrombocytosis\/leukocytosis<\/li><\/ul><\/li><\/ul>\n\n\n\n<p class=\"wp-block-paragraph\">Presents with arrhythmias, muscle weakness, decreased deep tendon reflexes, diarrhea, acidosis.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">ECG changes: T-wave peaking, P-wave flattening, PR prolongation, wide QRS complex<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Treatment: Glucose and insulin. In severe hyperkalemia, give IV calcium (protect the heart).<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Others: beta agonist, resin (absorbs potassium in the GI), furosemide. Hemodialysis.<\/p>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Cystic disease<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Acquired cysts<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Congenital cysts<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Nephronophthisis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">MCKD<\/h4><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Renal tubular disease<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Renal tubular acidosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Other diseases<\/h4><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Hypokalemia<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Hyperkalemia<\/h3><\/div>","protected":false},"excerpt":{"rendered":"<p>Cystic disease A cyst is an epithelium-lined sac of fluid. They are commonly found incidentally in the kidney as solitary lesions. Cystic kidney disease is a group of conditions in which the kidney develops numerous cysts. They can be acquired or congenital. Acquired cysts Most common in patients with CKD, specifically those on dialysis for [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":685,"menu_order":12,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-8111","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.9 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Cystic renal disease, renal tubular disease, hypo- and hyperkalaemia &#8211; Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/nephrology\/cystic-renal-disease-renal-tubular-disease-hypo-and-hyperkalaemia\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"4 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/nephrology\\\/cystic-renal-disease-renal-tubular-disease-hypo-and-hyperkalaemia\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/nephrology\\\/cystic-renal-disease-renal-tubular-disease-hypo-and-hyperkalaemia\\\/\",\"name\":\"Cystic renal disease, renal tubular disease, hypo- and hyperkalaemia &#8211; 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