Renal-GU diseases and Neoplasms

renal diseases, glomerulonephropathies, nephrotic syndrome, polycystic kidney disease, simple cyst, horseshoe kidney, and renal neoplasms. Flashcards review renal cell carcinoma and Wilm’s tumor.

Name the spectrum of glomerular diseases associated with the Nephrotic Mechanism (Injury to podocytes, changed architecture, scarring, deposition of matrix or other elements)

Minimal change nephropathy, FSGS, SLE, IgA nephropathy, Anti-GBM disease, Small vasculitis, Diabetic nephropathy, MGN, Post-streptococcal glomerulonephritis, Membranous nephropathy

What does the Nephritic Spectrum consist of?

Asymptomatic Nephritic Syndrome, RPGN, active urine sediment Glomerular Syndrome, Hematuria lots of dysmorphic red cells indicating glomerular bleeding; severe inflammation gives RBC casts. Micro or macroscopic hematuria, Chronic Glomerular Disease, Acute kidney injury, Proteinuria 1-3 g/d Hematuria RBC casts Edema Hypertension, Late-stage GN, burned-out disease, Proteinuria 1-3 g/d Hematuria. Proteinuria +/- proteinuria (<1 g/d) Hematuria RBC casts Edema Hypertension Systemic symptoms out disease

What are the symptoms of Glomerulonephropathy overview - Nephritic Syndrome?

HTN, Cola-colored urine (hematuria), oliguria, peripheral edema, massive proteinuria

What are the symptoms of Glomerulonephropathy overview - Nephrotic syndrome?

Hypoalbuminemia, hyperlipidemia

List the features of the Nephrotic Spectrum

Asymptomatic Nephrotic Proteinuria Syndrome, Chronic Glomerular Disease, bland urine sediment may be a few dysmorphic red cells, but the main finding is protein in the urine and bland urine sediment Proteinuria > 300 mg/d, can even be as much as 10 g/d. Hypoalbuminemia +/-Hematuria Edema Hyperlipidemia (lipiduria: oval fat bodies and fatty casts in urine) Proteinuria HTN, Late-stage GN, burned-out disease

What is the treatment for Minimal Change Glomerulonephritis?

Supportive care + Prednisolone. Most respond well

What is the treatment Focal Segmental Glomerulosclerosis?

Steroids often ineffective. 50% Progress to Renal Failure

What is the treatment for Vasculitic Disorders - Wegeners Granulomatosis?

Steroids + Cyclophosphamide

What is the treatment for Vasculitic Disorders - Microscopic Polyangitis?

long term steroids +/- cytotoxic agents

What is the treatment for Vasculitic Disorders - Goodpastures Syndrome?

steroids +/- steroid sparing agents

What is the treatment for Post Infectious Glomerulonephritis?

Supportive treatment. Resolves over 2-4 weeks

What are the essentials of diagnosis for Nephritic syndrome?

Hematuria, dysmorphic red blood cells, red cell casts, and mild proteinuria. Dependent edema and hypertension. Acute renal insufficiency.

Name parts of the kidney.

Renal artery, Renal vein, Ureter, Renal Pelvis, Medulla, Renal Pyramids, Kidney Cortex, Interlobular Vein, Interlobular Artery.

List the Etiology / Pathophysiology for Glomerulonephritis

Immunoglobulin A (IgA) nephropathy, Membranoproliferative glomerulonephritis (MPGN), Rapidly progressive glomerulonephritis (RPGN), Lupus nephritis, Postinfectious GN, Hereditary nephritis (Alport’s syndrome)

List the Symptoms / Clinical History for Glomerulonephritis

Hypertension, Gross hematuria: tea colored. Edema: facial and peripheral. Shortness of breath due to fluid overload. Malaise, Lethargy, Anorexia, Increase in weight, Pain in the back and joints

Symptoms suggestive of individual Glomerulonephritis diseases:

Sore throat or skin infection 2-4 weeks earlier: PSGN. Malar rash, Raynaud’s phenomenon, alopecia, arthralgias: Lupus. Purpuric rash over the body: Vasculitis. Fever: PIGN. Hemoptysis: Anti-GBM. Rhinorrhea, sinus pain, cough, and URI tract symptoms: IgG nephropathy. Deafness: Alport’s

What are the diagnostic studies for Glomerulonephritis?

CBC c Diff, CMP: lytes, BUN, Cr, Throat/Skin swab: R/O Strep, Urinalysis (dipstick). Urine microscopy, Red cell casts mean bleeding into the nephron. White cell casts mean inflammation in the renal interstitium. Antistreptolysin O (ASO) titer, Antibody screen, 24h urine collection

What is the acute treatment plan for Poststreptococcal Glomerulonephritis?

Largely supportive and appropriate antibiotics are given to eradicate causative bacterial infection

What is the acute treatment plan for Bacterial endocarditis Glomerulonephritis?

Antibiotic therapy until causative infection is eliminated

What is the acute treatment plan for MPGN, RPGN, Anti-GBM disease, Vasculitis

Cytotoxic agents (Cytoxan-cyclophosphamide) Mainstay therapy, Lupus nephritis in conjunction with steroids, Vasculitis in conjunction with steroids

What should be monitored in patients with Nephritis?

Periodic assessment of renal function, along with examination of urinary sediment and assessment of urine protein excretion, should be performed in all patients with nephritis

What are the essential diagnoses for Nephrotic Syndrome?

Urine protein excretion >3.5grams/24hours, Hypoalbuminemia (serum albumin <3g/dL), Peripheral edema, Hyperlipidemia, lipiduria, and hypercoagulability

What is hyperlipidemia and lipiduria?

Increase in serum cholesterol and phospholipids, and lipiduria are typically components of the nephrotic syndrome. This is due to increased hepatic synthesis which may be triggered by the fall in plasma oncotic pressure

What is the Etiology / Pathophysiology of Nephrotic Syndrome?

Minimal change disease/Nils disease, Membranous nephropathy, Focal segmental glomerulus sclerosis. Systemic Disorders Amyloidosis, Diabetic nephropathy, HIV-associated nephropathy, Systemic lupus erythematosus (SLE), Hepatitis C infection, Idiopathic membranoproliferative

What are the specialized studies for Nephrotic Syndrome?

Hepatitis serology: hepatitis B and C infection, Antinuclear antibodies (ANA): in active SLE nephritis, Low C3 and C4 complement. Renal biopsy

During an acute treatment plan for nephrotic syndrome, treatment can be considered as:

Symptomatic, supportive, or specific to the underlying condition

During an acute treatment plan for nephrotic syndrome, what are some general considerations?

Edema may be controlled with diuretics but hypovolemia may precipitate renal failure, Dietary salt restriction is essential for managing edema

What are the essentials of diagnosis for Autosomal-Dominant Polycystic Kidney Disease?

Multiple cysts in bilateral kidneys; total number depends on age. Large, palpable kidneys on examination. Combination of HTN and abdominal mass suggestive of disease, Family history

What are the major symptoms of Autosomal-Dominant Polycystic Kidney Disease?

Dull persistent flank and abdominal pain (60% of cases), Gross hematuria (30% of cases), Renal stones, cyst infections, and retroperitoneal hemorrhage also occur

What are the diagnostic studies for Autosomal-Dominant Polycystic Kidney Disease?

Hematuria or proteinuria on urinalysis, CBC – usually high hematocrit seen compared to level of renal function, CMP: Elevated BUN and Cr levels

What is treatment aimed at during the Acute Treatment Plan for Autosomal-Dominant Polycystic Kidney Disease?

Preserving renal function as far as possible by careful control of blood pressure, prompt treatment of bladder and kidney infection, and treatment of symptoms such as pain and hematuria

What are the complications of Autosomal-Dominant Polycystic Kidney Disease?

Rupture of intracranial aneurysms, Cardiac valvular abnormalities, Hepatic cysts

What are the essentials of diagnosis for Autosomal-Recessive Polycystic Kidney Disease?

Recessive pattern of transmission; parental consanguinity, Presentations for the neonatal period and infancy, Presentations for older children and adolescents

What are the typical symptoms and signs of Autosomal-Recessive Polycystic Kidney Disease?

Presents as bilateral abdominal masses in the first year of life, Hypertension and impaired urinary concentrating ability are common, Older children present with complications secondary to congenital hepatic fibrosis

What are the diagnostic studies for Autosomal-Recessive Polycystic Kidney Disease?

Ultrsound - Reveals enlarged kidneys with increased echogenicity, Spherical cysts may mimic appearance of ADPKD

What are the anatomical/physiological aspects of Autosomal-Recessive Polycystic Kidney Disease?

Smooth external surface, Distal tubules and collecting ducts are dilated into elongated cysts which becomes more spherical as patient ages

How is Hepatic fibrosis treated as a complication of Autosomal-Recessive Polycystic Kidney Disease?

May necessitate liver transplantation

What is a simple renal cyst?

Common finding, account for 65-70% of all renal masses. Generally found in the outer cortex. Cyst fluid is consistent with the plasma glomerular filtrate. Often an incidental finding on US, or CAT scan. Asymptomatic. Benign (must R/O renal cell CA)

When is it thought that a Horseshoe Kidney Fusion occurs?

Fusion is thought to occur before the kidneys ascend from the pelvis to their normal dorsolumbar position

If a horseshoe kidney is detected antenatally, what evaluation needs to be performed?

Ultrasound postnatal should be performed to confirm the diagnosis and define the underlying anatomy including whether hydronephrosis is present

What risks do patients with renal ectopy or fused kidneys have?

Patients with renal ectopy or fused kidneys are at increased risk for other anomalies, especially genitourinary abnormalities such as vesicoureteral reflux (VUR)

What are the essential diagnoses for Renal Cell Carcinoma?

Gross or microscopic hematuria, Flank pain or mass in some patients, Systemic symptoms such as fever, weight loss, may be prominent, Solid renal mass on x-ray imaging

Where does Renal cell carcinoma originate?

Renal cell carcinoma originates from the proximal tubule cells

What are the symptoms and signs associated with Renal Cell Carcinoma?

Gross or microscopic hematuria in 60%, Flank pain in 30%, Abdominal mass, Metastatic disease: cough, bone pain, fever, weight loss, Paraneoplastic symptoms:

List the imaging studies for Renal Cell Carcinoma

CT scan, Ultrasonography, Bone scan, Chest x-ray

What is the surgical care for Renal Cell Carcinoma?

Surgical resection (radical nephrectomy; > 7cm) remains the only known effective treatment for localized renal cell carcinoma

What is the Etiology / Pathophysiology of Wilms' tumor?

Wilms tumor is thought to be caused by alterations of genes responsible for normal genitourinary development

What is the most common presentation of Wilms' tumor?

The most common presentation of Wilms tumor is the presence of an asymptomatic abdominal mass.

What are the imaging studies for Wilms' tumor?

Renal ultrasonography, CT scanning, Chest radiography

What is the usual approach to treating Wilms' tumor?

Nephrectomy followed by chemotherapy with or without postoperative radiotherapy

Approximately what percentage of diagnosed children survive with current multimodality therapy for Wilms tumor?

80-90%