thursday tutorial

Here’s a very brief summary of what was discussed during the tutorial we had on Thursday. I have taken the liberty to consult e-med for parts that was discussed but didn’t know what was going on (which is, MOST of the time.) enjoy.

renal

Cystic renal dysplasia

Absence of normal parenchyma of kidney

Associated with Wilm’s

Not compatible with life

horse-shoe kidney

Effects come when ureters lie behind the joined pole of kidney

Obstructive nephropathy

Most of the time it lies in front so its okay

double ureters

Can have partial hydronephrosis

Normally benign

APKD

Why is it a problem - renal cell carcinoma

The main feature of ADPKD is bilateral progressive cystic dilation of the renal tubules, which may lead to end-stage renal disease (ESRD). Hepatic cysts, cerebral aneurysms, and cardiac valvular abnormalities also may occur.

Juvenilile Cystic KD

Complication - why not compatible with life?

Hepatic fibrosis

Lung fibrosis

All patients with autosomal recessive polycystic kidney disease have congenital hepatic fibrosis (CHF), which may have more severe clinical manifestation than the renal disease.

The CHF results from malformation of the developing ductal plate.

The liver biopsy findings reveal enlarged, fibrotic portal tracts and hyperplastic, dilated, and dysgenetic biliary ducts with normal hepatocytes. The ductules can show true cystic changes, and, when the changes are macroscopic, autosomal recessive polycystic kidney disease can be indistinguishable from Caroli disease. The portal hypertension secondary to the CHF can be clinically debilitating, with splenomegaly, varices, and GI hemorrhage.

tubulointerstitial diseases

pyelonephritis acute and chronic

·         Depressed scars - chronic pyelonephritis. Granular contracted kidney of pyelonephritis

·         What are the histologic differences in chronic pyelonephritis?

o   Tubulointerstitial infiltration by chronic inflammatory cells (lymphocytes)

o   Thyroidisation of tubules

o   Glomeruli usually spared - until renal failure

o   Colloidlike material pas+

obstructive uropathy

staghorn calculus

hydronephrosis

Hydronephrosis -> pus collect ->pyelonephrosis

Hydroureter

bph

Symptoms often attributed to BPH can be caused by other disease processes, and a history and physical examination are essential in ruling out other etiologies of (lower urinary tract symptoms (LUTS) (see Diagnostic Considerations).

·         Urinary frequency - The need to urinate frequently during the day or night (nocturia), usually voiding only small amounts of urine with each episode

·         Urinary urgency - The sudden, urgent need to urinate, owing to the sensation of imminent loss of urine without control

·         Hesitancy - Difficulty initiating the urinary stream; interrupted, weak stream

·         Incomplete bladder emptying - The feeling of persistent residual urine, regardless of the frequency of urination

·         Straining - The need strain or push (Valsalva maneuver) to initiate and maintain urination in order to more fully evacuate the bladder

·         Decreased force of stream - The subjective loss of force of the urinary stream over time

·         Dribbling - The loss of small amounts of urine due to a poor urinary stream

Diagnostics

·         DRE

o   The digital rectal examination (DRE) is an integral part of the evaluation in men with presumed BPH.

·         Prostate-Specific Antigen

o   Rising levels of PSA in serum are associated with prostate cancer. The PSA level also tends to rise in men with benign prostatic hyperplasia (BPH) and is a good marker for prostate volume. PSA levels are usually elevated in men with acute bacterial prostatitis.

·         Ultrasonography

o   A more precise volumetric determination can be made using transrectal ultrasonography (TRUS) of the prostate

Gliesen’s grading of prostatic cancer

prostate cancer

prostate cancer staging

renal cell carcinoma

There is an association of renal cell carcinoma with several clinical syndromes such as Von Hippel Lindau Disease, Adult Polycystic Kidney Disease, and Acquired Renal Cystic Disease.  Renal Cell Carcinoma has also been reported to run in certain families (a familial type).

clear cell carcinoma

Smoking, obesity, and hypertension are the 3 most well-established risk factors associated with development of sporadic renal cell carcinoma (RCC).

The main inherited disorder predisposing to development CCRCC is von Hippel-Lindau (VHL) disease, which involves a germline mutation of the VHL gene at chromosome 3p25. Affected individuals are susceptible to tumors of multiple organ systems, including cysts and tumors of the kidney, which occur in 25-45% of cases with a mean age at onset of 40 years. The renal tumors are frequently multifocal and/or bilateral and are always of the clear cell renal cell carcinoma (CCRCC) histologic type

wilms tumour

·         Wilms tumors can arise…

o   sporadically

o    in association with genetic syndromes,

§  Beckwith-Wiedemann syndrome (macroglossia, gigantism, and umbilical hernia)

§  Hemihypertrophy

§  Congenital aniridia

§  Wilms tumor, aniridia, genitourinary malformations, and mental retardation (WAGR syndrome)

§  Denys-Drash syndrome (Wilms tumor, pseudohermaphroditism, and glomerulopathy)

§  Trisomy 18 mutation

o   Familial

·         Presentation

o   Wilms tumor is diagnosed at a mean age of 3.5 years. The most common feature at presentation is an abdominal mass.

§  Abdominal pain occurs in 30%-40% of cases. Other signs and symptoms of Wilms tumor includehypertension, fever caused by tumor necrosis, hematuria, and anemia.

o   Major congenital anomalies include

§  genitourinary anomalies (WAGR and Denys-Drash syndromes, 5% of cases),

§  ectopic solitary horseshoe kidney,hypospadias and cryptorchidism, hemihypertrophy and organomegaly (Beckwith-Wiedemann syndrome, 2% of cases); and

§  aniridia (1% of cases).

§  Children with such syndrome anomalies should undergo periodic testing for Wilms tumor. Ultrasonography of the kidneys (once or twice per year) is a good screening tool.

·         Dwarfing of kidney

WAGR WT related syndromes

glomerulonephritis

Know what is common in adults / children

Adults

·         Membranous - 40%

·         Minimal change disease - 15%

·         Focal glomerulo sclerosis - 15%

·         Membranoproliferative - 7%

·         Mesangioproliferative - 5%

Children

·         Minimal change - 76% of nephrotic children

·         IgA nephropathy

·         Post-streptococcal

Rapidly progressive - why important to diagnose? - because treatment for severe and acute GN, is Dialysis and Transplantation. - RPGN patients frequently recur.

Diabetic Nephropathy - Kimmestel Wilson

Kimmelstiel-Wilson disease is a kidney condition associated with long-standing diabetes. It affects the network of tiny blood vessels (the microvasculature) in the glomerulus, a key structure in the kidney that is composed of capillary blood vessels and which is critically necessary for the filtration of the blood. Features of Kimmelstiel-Wilson disease include the nephrotic syndrome with excessive filtration of protein into the urine (proteinuria), high blood pressure (hypertension), and progressively impaired kidney function. When severe, Kimmelstiel-Wilson disease leads to kidney failure, end-stage renal disease, and the need for chronic kidney dialysis or a kidney transplant. The disorder is named for Paul Kimmelstein (1900-70), a German-born pathologist in the U.S., and Clifford Wilson (1906-), an English physician. It is also known as Kimmelstiel-Wilson syndrome or intercapillary glomerulonephritis.

DGSF

endocrine

pituitary adenoma

Sheehan's syndrome - when and what does occur?

parathyroid gland adenoma /hypo

hyperthyrodism

What are hurtle cells ? askanazi cells?

Papillary carcinoma of thyroid - cellular characteristics


Cusing's Syndrome - causes, characteristics