NEPHROLOGY Flashcards

Question

Complications Nephrotic Syndrome

Answer 1

↑ risk of infection due to urinary immunoglobulin loss * ↑ risk of thromboembolism related to loss of antithrombin III and plasminogen in the urine * Hyperlipidemia * Hypocalcemia (vitamin D and binding protein lost in urine) * Acute renal failure could be due to thrombotic renal veins it comes with lion pain and hematuria.

Answer 2

Presentation: proteinuria / nephrotic syndrome / CRF * Cause: infections, rheumatoid drugs, malignancy * 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRF

Answer 3

* Typically young adult with hematuria following an URTI * Associated with Henoch-Schonlein purpura * Mesangial hypercellularity (mesangioproliferative)

Answer 4

Classical post-streptococcal glomerulonephritis in child * Presents as nephritic syndrome / ARF * Most common form of renal disease in SLE (IV)

Answer 5

Typically a child with nephrotic syndrome (accounts for 80%) * Causes: Hodgkin's, NSAIDs * Good response to steroids

Answer 6

May be idiopathic or secondary to HIV, heroin * Presentation: proteinuria / nephrotic syndrome / CRF

Answer 7

Rapid onset, often presenting as ARF * Causes include Goodpasture's, ANCA positive vasculitis (e.g. Wegener's granulomatosis)

Answer 8

Type 1: cryoglobulinemia, hepatitis C → associated with low C4 * Type 2: partial lipodystrophy → associated with low C3

Answer 9

Post-streptococcal glomerulonephritis * Subacute bacterial endocarditis * Systemic lupus erythematosus * Mesangiocapillary glomerulonephritis

Answer 10

Renal biopsy demonstrates: * Sub-epithelial immune complex (mainly IgG and C3) deposition in the glomerulus * Electron microscopy: the basement membrane is thickened with sub-epithelial electron dense deposits (IgG, C3)

Answer 11

Idiopathic * Infections: hepatitis B, malaria * Malignancy: lung cancer, lymphoma, leukemia * Drugs: gold, penicillamine, NSAIDs * SLE (class V disease)

Answer 12

Immunosuppressant: steroids, cyclophosphamide, chlorambucil e.g. Ponticelli regime * BP control * Consider anticoagulation

Answer 13

Also called Berger's disease or mesangioproliferative glomerulonephritis * Commonest cause of glomerulonephritis worldwide * Pathogenesis unknown, ?Mesangial deposition of IgA immune complexes * Histology: mesangial hypercellularity, positive immunofluorescence for IgA & C3

Answer 14

* Post-streptococcal glomerulonephritis is associated with low complement levels * Main symptom in post-streptococcal glomerulonephritis is proteinuria (although hematuria can occur) * There is typically an interval between URTI and the onset of renal problems in post- streptococcal glomerulonephritis

Answer 15

Young ♂, recurrent episodes of Hematuria, usually painless (sometimes with no renal impairment) * Typically associated with mucosal infections e.g., URTI * Nephrotic range proteinuria is rare * Renal failure

Answer 16

Alcoholic cirrhosis * Celiac disease/dermatitis herpetiformis

Answer 17

25% of patients develop ESRF * Markers of good prognosis: frank hematuria * Markers of poor prognosis: ♂ gender, proteinuria (especially > 2 g/day), hypertension, smoking, hyperlipidemia, ACE genotype DD

Answer 18

Nearly always presents as nephrotic syndrome, accounting for 75% of cases in children and 25% in adults

Answer 19

prednisolone ACE inhibitors may be used to ↓ proteinuria in patients with heavy proteinuria or who have a slow response to prednisolone

Answer 20

Causes: majority of cases are idiopathic, but in around 10-20% a cause is found: * Drugs: NSAIDs, rifampicin * Hodgkin's lymphoma, NHL and thymoma * Infectious mononucleosis

Answer 21

* Nephrotic syndrome * Normotension - hypertension is rare * Hematuria is very rare * Highly selective proteinuria* * Renal biopsy: electron microscopy shows fusion of podocytes

Answer 22

* Majority of cases (80%) are steroid responsive * Cyclophosphamide is the next step for steroid resistant cases Prognosis is overall good, although relapse is common. Roughly: * 1/3 have just one episode * 2/3 have relapses: o 1/3 have infrequent relapses o 1/3 have frequent relapses which stop before adulthood

Answer 23

AKA membranoproliferative glomerulonephritis * May present as nephrotic syndrome, hematuria or proteinuria * Poor prognosis

Answer 24

Subendothelial immune deposits * Cause: Cryoglobulinemia (with low C4), hepatitis C

Answer 25

* ↓ HCO3- reabsorption in proximal tubule * Hypokalemia * Complications include osteomalacia * Causes include idiopathic, as part of Fanconi syndrome, Wilson's disease, cystinosis, outdated tetracyclines * Treat: Bicarb replacement + Thiazide diuretics

Answer 26

* Idiopathic * Secondary to other renal pathology e.g. IgA nephropathy, reflux nephropathy * HIV * Heroin * Alport's syndrome * Sickle-cell

Answer 27

is usually inherited in an X-linked dominant pattern*. It is due to a defect in the gene which codes for type IV collagen resulting in an abnormal glomerular-basement membrane (GBM). The disease is more severe in ♂s with ♀s rarely developing renal failure A favorite question in the MRCP is an Alport's patient with a failing renal transplant. This may be caused by the presence of anti-GBM antibodies leading to a Goodpasture's syndrome like picture

Answer 28

* Progressive renal failure * Bilateral sensorineural deafness * Lenticonus: protrusion of the lens surface into the anterior chamber * Retinitis pigmentosa

Answer 29

Risk factors * Dehydration * Hypercalciuria, hyperparathyroidism, hypercalcemia * Cystinuria NOT CYSTINOSIS * High dietary oxalate * Renal tubular acidosis * Medullary sponge kidney, polycystic kidney disease * Beryllium or cadmium exposure

Answer 30

Drugs that promote calcium stones: loop diuretics, steroids, acetazolamide, theophylline * Thiazides can prevent calcium stones (↑ distal tubular calcium resorption)

Answer 31

stag-horn calculi i

Answer 32

Diclofenac 75 mg by intramuscular injection is the analgesia of choice for renal colic*. A second dose can be given after 30 minutes if necessary. (*PR diclofenac is an alternative)

Answer 33

Calcium stones * High fluid intake * Low animal protein, low salt diet (a low calcium diet has not been shown to be superior to a normocalcemic diet) * Thiazide diuretics (↑ distal tubular calcium resorption) * Stones < 5 mm will usually pass spontaneously * Lithotripsy, nephrolithotomy may be required

Answer 34

Cholestyramine ↓ urinary oxalate secretion * Pyridoxine ↓ urinary oxalate secretion

Answer 35

Stage 1 * Hyperfiltration: ↑ in GFR (60-140 ml/min/1.73m2) * May be reversible Stage 2 (silent or latent phase) * Most patients do not develop microalbuminuria for 10 years * GFR remains elevated Stage 3 (incipient nephropathy) * Microalbuminuria (albumin excretion of 30 - 300 mg/day, dipstick negative) Stage 4 (overt nephropathy) * Persistent proteinuria (albumin excretion > 300 mg/day, dipstick positive) * Hypertension is present in most patients * Histology shows diffuse glomerulosclerosis and focal glomerulosclerosis (kimmelstiel-wilson nodules) Stage 5 * End-stage renal disease, GFR typically < 10ml/min * Renal replacement therapy needed

Answer 36

Screening * All patients should be screened annually * Albumin:Creatinine ratio (ACR) in early morning specimen * ACR > 2.5 = microalbuminuria

Answer 37

Dietary protein restriction * Tight glycemic control * BP control: aim for < 130/80 mmHg * Benefits independent of blood pressure control have been demonstrated for ACE inhibitors and angiotensin II receptor blockers - these may be used alone or in combination * Control dyslipidemia e.g. Statins

Answer 38

is an important marker of chronic kidney disease, especially for diabetic nephropathy. NICE recommend using the albumin:creatinine ratio (ACR) in preference to the protein:creatinine ratio (PCR) when identifying patients with proteinuria as it has greater sensitivity. For quantification and monitoring of proteinuria, PCR can be used as an alternative, although ACR is recommended in diabetics. Urine reagent strips are not recommended unless they express the result as an ACR

Answer 39

Urinary tract infection * Menstruation * Vigorous exercise * Sexual intercourse

Answer 40

describes a generalized disorder of renal tubular transport resulting in: * Type 2 (proximal) renal tubular acidosis * Aminoaciduria * Glycosuria * Phosphaturia * Osteomalacia

Answer 41

hyperchloremic metabolic acidosis (normal anion gap)

Answer 42

nability to generate acid urine (secrete H+) in distal tubule * Hypokalemia * Complications include nephrocalcinosis and renal stones * Causes include idiopathic, RA, SLE, Sjogren's

Answer 43

is not actually a tubular disorder at all nor does it have a clinical picture similar to the other RTAs. It was included in the classification of RTA as it is associated with a mild (normal anion gap) metabolic acidosis due to a physiological ↓ in proximal tubular ammonium excretion.

Answer 44

Features: * Results from inherited carbonic anhydrase II deficiency. * Mutations in the gene encoding this enzyme give rise to: o Autosomalrecessivesyndromeofosteopetrosis o Renal tubular acidosis o Cerebral calcification o Mental retardation. * 70% of the reported cases are from the Magreb region of North Africa Type 3 is rarely discussed

Answer 45

Urine sodium < 20 mmol/L vs > 30 mmol/L

Answer 46

Causes * Chronic analgesia use * Sickle cell disease * TB * Acute pyelonephritis * Diabetes Mellitus

Answer 47

Fever, loin pain, hematuria * IVU (intravenous urogram or IVP for pyelogram) - papillary necrosis with renal scarring - 'cup & spill'

Answer 48

Causes * Partially treated UTI * Urethritis e.g. Chlamydia, TB and ureaplasma urealyticum * Renal tuberculosis * Renal stones * Appendicitis * Bladder/renal cell cancer * Adult polycystic kidney disease * Analgesic nephropathy

Answer 49

* More common in middle-aged men * Smoking * Von hippel-lindau syndrome * Tuberous sclerosi

Answer 50

* Classical triad: hematuria, loin pain, abdominal mass * Pyrexia of unknown origin * Left varicocele (due to occlusion of left testicular vein) * Endocrine effects: may secrete erythropoietin (polycythemia), parathyroid hormone (hypercalcemia), renin, ACTH * 25% have metastases at presentation

Answer 51

Radical nephrectomy for confined disease * α-interferon and interleukin-2 have been used to ↓ tumor size and also treat patients with metastases * Receptor tyrosine kinase inhibitors (e.g. Sorafenib, sunitinib) have been shown to have superior efficacy compared to interferon-α

Answer 52

is one of the most common childhood malignancies. It typically presents in children less than 5 years of age, with a median age of 3 years.

Answer 53

eatures * Abdominal mass (most common presenting feature) * Painless hematuria * Flank pain * Other features: anorexia, fever * Unilateral in 95% of cases * Metastases are found in 20% of patients

Answer 54

Beckwith-Wiedemann syndrome is a inherited condition associated with organomegaly, macroglossia, abdominal wall defects, Wilm's tumor and neonatal hypoglycemia * As part of WAGR syndrome: Wilms Aniridia, Genitourinary malformations, mental Retardation. It results from a deletion on chromosome 11 resulting in the loss of several genes. * Hemihypertrophy * Around one-third of cases are associated with a mutation in the WT1 gene on chromosome 11

Answer 55

hypertension, chronic renal failure or 'flash' pulmonary edema.FMD is more common in young women and characteristically has a 'string of beads' appearance on angiography.

Answer 56

MR angiography is now the investigation of choice * CT angiography * Conventional renal angiography is less commonly performed used nowadays, but may still have a role when planning surgery

Answer 57

Massive proteinuria * Normal or large kidneys * Focal segmental glomerulosclerosis with focal or global capillary collapse on renal biopsy * Elevated urea and creatinine * Normotension

Answer 58

IgG deposits on renal biopsy * Anti-GBM antibodies

Answer 59

a type II hypersensitivity reaction autoimmune disease triggered when the patient’s immune system anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen.

Answer 60

Features * Pulmonary hemorrhage * Followed by rapidly progressive glomerulonephritis

Answer 61

Renal biopsy: linear IgG deposits along basement membrane * ↑ transfer factor secondary to pulmonary hemorrhages * Lung biopsy: accumulation of hemosidren laden macrophages with alveoli

Answer 62

generally seen in young children and produces a triad of: * Acute renal failure * Microangiopathic hemolytic anemia * Thrombocytopenia

Answer 63

Post-dysentery - classically E coli 0157:H7 ('verotoxigenic', 'enterohemorrhagic') * Tumors * Pregnancy * Cyclosporine, the Pill * SLE * HIV

Answer 64

* Full blood count: anemia, thrombocytopenia, fragmented blood film * U&E: acute renal failure * Stool culture

Answer 65

Management * Treatment is supportive e.g. Fluids, blood transfusion and dialysis if required * There is no role for antibiotics, despite the preceding diarrheal illness in many patients * The indications for plasma exchange in HUS are complicated. As a general rule plasma exchange is reserved for severe cases of HUS NOT associated with diarrhea

Answer 66

is an autosomal recessive condition caused by a disorder of phenylalanine metabolism. defect in phenylalanine hydroxylase, an enzyme which converts phenylalanine to tyrosine gene for phenylalanine hydroxylase is located on chromosome 12

Answer 67

Usually presents by 6 months e.g. With developmental delay * Child classically has fair hair and blue eyes * Learning difficulties * Seizures, typically infantile spasms * Eczema * 'Musty' odor to urine and sweat*

Answer 68

an autosomal recessive disorder characterized by the formation of recurrent renal stones. It is due to a defect in the membrane transport of cystine, ornithine, lysine, arginine (mnemonic = COLA) Genetics Recurrent renal stones * Are classically yellow and crystalline, appearing semi-opaque on x-ray

Answer 69

Chromosome 2: SLC3A1 gene, chromosome 19: SLC7A9

Answer 70

Diagnosis * Cyanide-nitroprusside test Management * Hydration * D-penicillamine * Urinary alkalinization

Answer 71

are autosomal recessive disease caused by deficiency of cystathione β- synthetase. This results in an accumulation of homocysteine which is then oxidized to homocysteine.

Answer 72

* Often patients have fine, fair hair * Musculoskeletal: may be similar to Marfan's - arachnodactyly etc * Neurological patients may have learning difficulties, seizures * Ocular: downwards dislocation of lens (Marfan has upward dislocation of lens) * ↑ Risk of arterial and venous thromboembolism except coronaries. * Also malar flush, livedo reticularis

Answer 73

* Rare inherited genetic disorder of phenylalanine and tyrosine metabolism * Autosomal recessive * Common in Slovakia and the Dominican Republic than in other countries. * Due to a defect in the enzyme homogentisate 1,2-dioxygenase, which participates in the degradation of tyrosine. As a result, a toxic tyrosine byproduct called homogentisic acid (or alkapton) accumulates in the blood and is excreted in urine in large amounts. Excessive homogentisic acid causes damage to cartilage (ochronosis, leading to osteoarthritis) and heart valves as well as precipitating as kidney stones.

Answer 74

The main symptoms of alkaptonuria are due to the accumulation of homogentisic acid in tissues. In the joints this leads to cartilage damage, specifically in the spine, leading to low back pain at a young age in most cases. Cartilage damage may also occur in the hip and shoulder. Joint replacement surgery (hip and shoulder) is often necessary at a relatively young age. Valvular heart disease, - progressive cases valve replacement may be necessary. Coronary artery disease may be accelerated in alkaptonuri ar wax exposed to air turns red or black

Answer 75

Risk factors * Age: around 50% of 50-year-old men will have evidence of BPH and 30% will have symptoms. Around 80% of 80-year-old men have evidence of BPH * Ethnicity: Black > White > Asian

Answer 76

Voiding symptoms (obstructive): weak or intermittent urinary flow, straining, hesitancy, terminal dribbling and incomplete emptying * Storage symptoms (irritative) urgency, frequency, urgency incontinence and nocturia * Post-micturition: dribbling * Complications: urinary tract infection, retention, obstructive uropathy

Answer 77

W atchful waiting * Medication: α-1 antagonists, 5 α-reductase inhibitors. The use of combination therapy was supported by the medical therapy of prostatic symptoms (MTOPS) trial * Surgery: transurethral resection of prostate (TURP)

Answer 78

↓ smooth muscle tone (prostate and bladder) * Considered first-line, improve symptoms in around 70% of men * Adverse effects: dizziness, postural hypotension, dry mouth, depression

Answer 79

* Block the conversion of testosterone to dihydrotestosterone (DHT), which induces BPH * Unlike α-1 antagonists causes a reduction in prostate volume and hence may slow disease progression. This however takes time and symptoms may not improve for 6 months. They may also ↓ PSA concentrations by up to 50% * Adverse effects: erectile dysfunction, ↓ libido, ejaculation problems, gynecomastia

Answer 80

* Age * ↑ fat diet * Family Hx * BPH is not a risk factor

Answer 81

Localized disease = T1/2 T1 - clinically unapparent disease: * If life expectancy < 10 years then watchful waiting * If life expectancy > 10 years then offer: o Radical prostatectomy o Radical radiotherapy T2 - palpable disease confined to prostate* Radical prostatectomy * Radical radiotherapy (often if older patient) Locally advanced disease (T3/4) * T3 = beyond prostatic capsule } * T4 = involves bladder neck or rectum * Most men will have occult mets Disseminated disease - hormonal therapy Radiotherapy

Answer 82

E.g. Goserelin (zoladex) is a synthetic GnRH agonist which provides negative feedback to the anterior pituitary. o Cover initially with anti-androgen to prevent rise in testosterone

Answer 83

common urological cancer with most cases being transitional cell carcinomas. It has a ♂:♀=3:1 with women generally having a worse prognosis than men. The most classical presentation is with total, gross, painless hematuria.

Answer 84

* Smoking * Occupational: aniline dyes used in printing and textile industry, rubber manufacture * Aromatic amines * Prior radiation treatment to the pelvis * Exposure to a urinary metabolite of cyclophosphamide (acrolein). * Schistosomiasis (S. hematobium infection) * Mutations on 17p13.1, the gene coding for p53, mutations of which are associated with high- grade bladder cancer * Mutation on 9p15 and 9p16, another tumor suppressor gene associated with low grade and superficial tumors. * Drugs: cyclophosphamide

NEPHROLOGY Flashcards

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