Last minute revision of Nephrology

 Important points of Nephrology to revise during last minute.

Horseshoe kidneyKidneys function normally. Associated withhydronephrosis (eg, ureteropelvic junction obstruction ) , renal stones, infection, hromosomal aneuploidy syndromes (eg, Turner syndrome; trisomies 13, 18, 21), and rarely renal cancer.
renal agenesisUnilateral agenesis leads to hypertrophy of the existing kidney; hyperfiltration , increases risk of renal failure later in life.Bilateral agenesis leads to oligohydramnios with lung hypoplasia, flat face with low set ears, and developmental defects of the extremities (Potter sequence );incompatible with life .
dysplastic kidneyThose with bilateral disease often have other severe deformities or polysystemic malformation syndromes. In bilateral cases, the newborn has the classic characteristic of Potter’s syndrome .
polycystic kidney diseaseADPKD : presents in young adults as hypertension (due to increased renin), hematuria, and worsening renal failure. Associated with berry aneurysm, hepatic cysts, and mitral valve prolapsed .ARPKD : presents in infants as worsening renal failure and hypertension; newborns may present with Potter sequence . Associated with congenital hepatic fibrosis (leads to portal hypertension) and hepatic cysts .
medullary cystic kidney diseaseParenchymal fibrosis results in shrunken kidneys and worsening renal failure.
potter sequence (syndrome )Oligohydramnios → compression of developing fetus → limb deformities, facial anomalies (eg, low-set ears and retrognathia , flattened nose), compression of chest and lack of amniotic fluid aspiration into fetal lungs → pulmonary hypoplasia (cause of death).
duplex collecting systemStrongly associated with vesicoureteral reflux and/or ureteral obstruction , ↑ risk for UTIs.
Congenital solitary functioning kidneyMajority asymptomatic with compensatory hypertrophy of contralateral kidney, but anomalies in contralateral kidney are common.
Posterior urethral valvesdiagnosed prenatally by hydronephrosis and dilated or thick-walled bladder on ultrasound. Most common cause of bladder outlet obstruction in male infants.
hartnup diseasemanifests during infancy with variable clinical presentation: failure to thrive, photosensitivity, intermittent ataxia, nystagmus, and tremor.
Acute renal failureMetabolic AcidosisDyslipidemia (especially triglycerides) HyperkalemiaUremia—clinical syndrome marked by BUN:Nausea and anorexia PericarditisAsterixis Encephalopathy Platelet dysfunctionNa+/H2O retention (HF, pulmonary edema, hypertension)Growth retardation and developmental delay Erythropoietin failure (anemia)Renal osteodystrophy .Prerenal : Due to ↓ RBF (eg, hypotension)Postrenal : outflow obstruction (stones, BPH, neoplasia, congenital anomalies). Develops only with bilateral obstruction .Acute tubular necrosis : Generally due to acute tubular necrosis or ischemia/toxins; less commonly due to acute glomerulonephritis(eg, RPGN, hemolytic uremic syndrome ) or acute interstitial nephritis. 3 stages :Inciting eventMaintenance phase—oliguric; lasts 1–3 weeks; risk of hyperkalemia, metabolic acidosis, uremiaRecovery phase—polyuric; BUN and serum creatinine fall; risk of hypokalemia
Chronic renal failure Uremia-Increased nitrogenous waste products in blood (azotemia) result in nausea, anorexia, pericarditis, platelet dysfunction, encephalopathy with asterixis, and deposition of urea crystals in skin.Salt and water retention with resultant hypertensionHyperkalemia with metabolic acidosisAnemia due to decreased erythropoietin production by renal peritubular interstitial cellsHypocalcemia due to decreased 1-alpha-hydroxylation of vitamin D by proximal renal tubule cells and hyperphosphatemiaRenal osteodystrophy due to secondary hyperparathyroidism , osteomalacia, and osteoporosis
Acute interstitial nephritisPresents as oliguria, fever, and rash days to weeks after starting a drug; eosinophils may be seen in urine.
Renal papillary necrosisPresents with gross hematuria and flank pain
Renal tubular defectsFanconi : result in metabolic acidosis (proximal renal tubular acidosis).Barter : Results in hypokalemia and metabolic alkalosis with hypercalciuria , Presents similarly to chronic loop diuretic use.Gitelman : Leads to hypokalemia, hypomagnesemia, metabolic alkalosis, hypocalciuria. Similar to using life-long thiazide diureticsLiddle : Results in hypertension, hypokalemia, metabolic alkalosis, ↑aldosterone. Presents like hyperaldosteronismSyndrome of apparent mineralocorticoid excess : hypertension, hypokalemia, metabolic alkalosis. Low serum aldosterone levels.
Metabolic acidosischest pain, palpitations, headache, altered mental status such as severe anxiety due to hypoxia, decreased visual acuity ,nausea , vomiting , abdominal pain, altered appetite and weight gain ,muscle weakness, bone pain, and joint pain. Those in metabolic acidosis may exhibit deep, rapid breathing called Kussmaul respirations which is classically associated with diabetic ketoacidosis. Extreme acidemia leads to neurological and cardiac complications:Neurological: lethargy, stupor, coma, seizures .Cardiac: arrhythmias (ventricular tachycardia) and decreased response to epinephrine, both leading to hypotension .
Metabolic alkalosisMetabolic alkalosis : slower breathing, or hypoventilation (respiratory compensation) .
Renal tubular acidosisDistal type 1 : metabolic acidosis. Associated with hypokalemia, ↑ risk for calcium phosphate kidney stones .Proximal type 2 : subsequent metabolic acidosis.Associated with hypokalemia, ↑ risk for hypophosphatemic rickets.Hyperkalemic type 4 : Hypoaldosteronism → hyperkalemia .
Nephrotic syndromemassive proteinuria (> 3.5 g/day) with hypoalbuminemia, resulting edema, hyperlipidemia. Frothy urine with fatty casts. Associated with hypercoagulable state (eg, thromboembolism) due to antithrombin (AT) III loss in urine and ↑ risk of infection (due to loss of immunoglobulins in urine and soft tissue compromise by edema).
Nephritic syndromehematuria and RBC casts in urine. Associated with azotemia, oliguria, hypertension (due to salt retention), proteinuria (< 3.5 g/day) but in severe cases may be in nephrotic range. Poststreptococcal glomerulonephritis : Occurs ~ 2–4 weeks after group A streptococcal infection of pharynx or skin. Resolves spontaneously. Presents with peripheral and periorbital edema, cola-colored urine, hypertension. Rapidly progressive glomerulonephritis : Rapidly deteriorating renal function (days to weeks).
good pasture syngeneralized symptoms like malaise, weight loss, fatigue, fever, and chills are also common, as are joint aches and pains . lung symptoms : coughing up blood, chest pain (in less than 50% of cases overall), cough , and shortness of breath , Kidney symptoms usually include blood in the urine, protein in the urine , unexplained swelling of limbs or face, high amounts of urea in the blood, and high blood pressure .
granlomatosis whith polyangiitis ( Wegener granulomatosis )In general, rhinitis is the first sign in most people.•             Kidney: rapidly progressive glomerulonephritis (75%), leading to chronic kidney failure•             Upper airway, eye and ear disease:•             Nose: pain, stuffiness, nosebleeds, rhinitis, crusting , saddle-nose deformity due to a perforated septum•             Ears: conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss (unclear mechanism)•             Oral cavity: strawberry gingivitis, underlying bone destruction with loosening of teeth, non-specific ulcerations throughout oral mucosa•             Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis•             Trachea: subglottal stenosis•             Lungs: pulmonary nodules (referred to as “coin lesions”), infiltrates (often interpreted as pneumonia), cavitary lesions, pulmonaryhaemorrhage causing haemoptysis, and rarely bronchial stenosis.•             Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis•             Skin: nodules on the elbow, purpura, various others (see cutaneous vasculitis)•             Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex•             Heart, gastrointestinal tract, brain, other organs: rarely affected.
Microscopic PolyangiitisGeneralised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common. The skin may show rashes, swelling, ulcers, and lumps. palpable purpura and livedo reticularis can occur in some patients. Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness (peripheral neuropathy). Central nervous system involvement may cause strokes or seizures. Kidney involvementcan produce varying degrees of kidney failure, such as hypertension, edema, oliguria,and uremia. Involvement of the arteries of the heart may cause a heart attack , heart failure, and inflammation of the sac around the heart (pericarditis).
IG A nephropathy ( berger )Episodic gross hematuria that occurs concurrently with respiratory or GI tract infections .
Membranoproliferative glomerulonephritispresent as nephrotic syndrome and nephritic syndrome concurrently.
Diffuse proliferative glomerulonephritispresent as nephrotic syndrome and nephritic syndrome concurrently.
Cystitissuprapubic pain, dysuria, urinary frequency, urgency. Systemic signs (eg, high fever, chills) are usually absent.
PyelonephritisAcute : fevers, flank pain (costovertebral angle tenderness ), nausea/vomiting, chills. Chronic : Coarse, asymmetric corticomedullary scarring, blunted calyx. A perinephric abscess (infection around the kidney) and/or pyonephrosis may develop in severe cases of pyelonephritis . Xanthogranulomatous pyelonephritis : widespread kidney damage due to granulomatous tissue containing foamy macrophages
Diffuse cortical necrosisacute kidney failure. associated with obstetric catastrophes such as abruptio placentae and septic shock .
Renal osteodystrophyHypocalcemia, hyperphosphatemia , and failure of vitamin D hydroxylation associated with chronic renal disease → 2° hyperparathyroidism. Causes subperiosteal thinning of bones .
Hydronephrosisoccurs acutely with sudden onset (as caused by a kidney stone) can cause intense pain in the flank area (between the hips and ribs). Historically, this type of pain has been described as “Dietl’s crisis”. Conversely, hydronephrosis that develops gradually will generally cause either attacks of a dull discomfort or no pain. Nausea and vomiting may also occur. An obstruction that occurs at the urethra or bladder outlet can cause pain and pressure resulting from distension of the bladder. Blocking the flow of urine will commonly result in urinary tract infections which can lead to the development of additional stones, fever, and blood or pus in the urine. If complete obstruction occurs, kidney failure may follow .
Urinary incontinenceStress incontinence : leak with ↑ intra-abdominal pressure (eg, sneezing, lifting).Urgency incontinence : leak with urge to void immediately .Mixed incontinence : Features of both stress and urgency incontinence.Overflow incontinence : leak with overfilling.
AngiomyolipomaHamartoma comprised of blood vessels, smooth muscle, and adipose tissue .
Renal cell carcinomahematuria, palpable mass, 2° polycythemia, flank pain, fever, weight loss. Invades renal vein (may develop varicocele if left sided) then IVC and spreads hematogenously; metastasizes to lung and bone.
Wilms tumor ( nephroblastoma )large, palpable, unilateral flank mass and/or hematuria.
Renal oncocytomapainless hematuria, flank pain, abdominal mass.
Lower urinary tract carcinomaUrothelial ( transitional cell carcinoma ) : painless hematuria .Squamous cell carcinoma : painless hematuriaAdenocarcinoma : painless hematuria
Last minute revision of Nephrology
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