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Cystitis Cystitis is a urinary bladder inflammation that can result from any one of a number of distinct syndromes.


for years before they are told that their urine cultures are negative. Antibiotics are not used in the treatment of IC. The cause of IC is unknown, though some suspect it may be autoimmune where the immune system attacks the bladder. Several therapies are now available.

It is most commonly caused by

a bacterial infection in which case it is referred to as a urinary tract infection. Signs and symptoms

Eosinophilic cystitis is a rare form of cystitis that is diagnosed via biopsy. In these cases, the

Pressure in the lower pelvis Painful urination (dysuria) Frequent urination (polyuria) or urgent need to urinate (urinary urgency)

bladder wall is infiltrated with a high number of eosinophils. The cause of EC may be attributed to infection by Schistosoma haematobium or by certain medications in afflicted children. Some consider it a form of

Need to urinate at night (nocturia) interstitial cystitis. Urine that contains traces of blood (haematuria) Dark, cloudy or strong-smelling urine Pain above the pubic bone, or in the lower back or abdomen

Hemorrhagic cystitis, can occur as a side effect of cyclophosphamide, ifosfamide, and radiation therapy. Radiation cystitis, one form of hemorrhagic cystitis is a rare consequence of

Feeling unwell, weak or feverish


patients undergoing radiation therapy for the treatment of cancer. Several adenovirus serotypes have been associated with an acute, self-limited hemorrhagic cystitis, which occurs primarily in boys. It is characterized by hematuria, and virus

Subtypes There are several medically distinct types of cystitis, each having a unique etiology and therapeutic approach:

Traumatic cystitis is probably the most common form of cystitis in the female, and is due to bruising of the bladder, usually by abnormally forceful sexual intercourse. This is often followed bybacterial cystitis, frequently by coliform bacteria being transferred from the bowel through the urethra into the bladder.

can usually be recovered from the urine.

In sexually active women the most common cause of urinary tract infection is from E. coli and Staphylococcus saprophyticus.

Cystitis cystica is a chronic cystitis glandularis accompanied by the formation of cysts. This disease can cause chronic urinary tract infections. It appears as small cysts filled with fluid and lined by one or more layers of epithelial cells.These are due to hydropic degeneration in center of Brunn's nests

Interstitial cystitis (IC) is considered more of an injury to the bladder resulting in constant irritation and rarely involves the presence of infection. IC patients are often misdiagnosed with UTI/cystitis

Diagnostic approach

In healthy individuals the ureters enter the urinary bladder obliquely and run submucosally for some

A urinalysis may reveal white blood cells (WBCs) or red blood cells (RBCs).

distance. This, in addition to the ureter's muscular attachments, helps secure and support them posteriorly. Together these features produce a valvelike effect that occludes the ureteric opening during storage and voiding of urine. In people with VUR, failure of this mechanism occurs, with resultant retrograde flow of urine. Hardikar syndrome can include Vesicoureteral reflux, Hydronephrosis, cleft lip and palate, Intestinal obstruction and other symptoms.

A urine culture (clean catch) or catheterized urine specimen may be performed to determine the type of bacteria in the urine and the appropriate antibiotic for treatment.

Treatment Treatment depends on the underlying cause.

Vesicoureteral reflux Primary VUR Insufficient submucosal length of the ureter relative to Vesicoureteral reflux (VUR) is an abnormal movement of urine from the bladder into ureters or kidneys. Urine normally travels from the kidneys via the ureters to the bladder. In vesicoureteral reflux the direction of urine flow is reversed (retrograde). Secondary VUR Symptoms Vesicoureteral reflux may present before birth as prenatal hydronephrosis, an abnormal widening of the ureter or with a urinary tract infection or acute pyelonephritis. Symptoms such as painful urination or renal colic/flank pain are not symptoms associated with vesicoureteral reflux. Newborns may be lethargic with faltering growth, while infants and young children typically present with pyrexia, dysuria, frequent urination, malodorous urine and GIT symptoms, but only when urinary tract infection is present as the initial presentation of VUR. Causes Functional: Bladder instability, neurogenic bladder and non-neurogenic neurogenic bladder Urinary tract In this category the valvular mechanism is intact and healthy to start with but becomes overwhelmed by raised vesicular pressures associated with obstruction, which distorts the ureterovesical junction. The obstructions may be anatomical or functional. Secondary VUR can be further divided into anatomical and functional groups as follows: Anatomical: Posterior urethral valves; urethral or meatal stenosis. These causes are treated surgically when possible. its diameter causes inadequacy of the valvular mechanism. This is precipitated by a congenital defect/lack of longitudinal muscle of the intravesical ureter resulting in an ureterovesicular junction (UVJ) anomaly.

infections may cause reflux due to the elevated pressures associated with inflammation.

Grade V gross dilatation of the ureter, pelvis and calyces; ureteral tortuosity; loss of papillary impressions

Resolution of functional VUR will usually occur if the precipitating cause is treated and resolved. Medical and/or surgical treatment may be indicated. Prevalence It has been estimated that VUR is present in more than 10% of the population. In children without urinary tract infections 17.2-18.5% have VUR, whereas in those with urinary tract infections the incidence may be as high as 70%. Age Younger children are more prone to VUR because of the relative shortness of the submucosal ureters. This susceptibility decreases with age as the length of the ureters increases as the children grow. In children under the age of 1 year with a urinary tract infection, 70% will have VUR. This number decreases to 15% by the age of 12. Sex
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The younger the age of the patient and the lower the grade at presentation the higher the chance of spontaneous resolution. Most (approx. 85%) of grade I & II cases of VUR will resolve spontaneously. Approximately 50% of grade III cases and a lower percentage of higher grades will also resolve spontaneously. Diagnosis VCUG demonstrating bilateral Grade III vesicoureteral reflux. The following procedures may be used to diagnose VUR:

Nuclear cystogram (RNC) Fluoroscopic voiding cystourethrogram (VCUG) Ultrasonic cystography Abdominal ultrasound

An abdominal ultrasound might suggest the presence Although VUR is more common in males antenatally, in later life there is a definite female preponderance with 85% of cases being female. International Classification of Vesicoureteral Reflux of VUR if ureteral dilatation is present; however, in many circumstances of VUR of low to moderate severity, the sonogram may be completely normal, thus providing insufficient utility as a single diagnostic test in the evaluation of children suspected of having

Grade I reflux into non-dilated ureter Grade II reflux into the renal pelvis and calyces without dilatation

VUR, such as those presenting with prenatal hydronephrosis or urinary tract infection (UTI). VCUG is the method of choice for grading and initial workup, while RNC is preferred for subsequent evaluations as there is less exposure to radiation. A high index of suspicion should be attached to any case a where a child presents with a urinary tract infection, and anatomical causes should

Grade III mild/moderate dilatation of the ureter, renal pelvis and calyces with minimal blunting of the fornices

Grade IV dilation of the renal pelvis and calyces with moderate ureteral tortuosity

be excluded. A VCUG and abdominal ultrasound should be performed in these cases Early diagnosis in children is crucial as studies have shown that the children with VUR who present with a UTI and associated acute pyelonephritis are more likely to develop permanent renal cortical scarring than those children without VUR, with an odds ratio of 2.8.

Medical Treatment Medical treatment entails low dose antibiotic prophylaxis until resolution of VUR occurs. Antibiotics are administered nightly at half the normal therapeutic dose. The specific antibiotics used differ with the age of the patient and include:

Thus VUR not only increases the frequency of

Amoxicillin or ampicillin - infants younger than 6 weeks

UTI's, but also the risk of damage to upper urinary structures. Treatment

Trimethoprim-sulfamethoxazole (co-trimoxazole) - 6 weeks to 2 months

After 2 months the following antibiotics are suitable: Medical treatment is the preferred mode of management, but surgical interventions may be necessary. Medical management is recommended in children with Grade I-III VUR as most cases will resolve spontaneously. A trial of medical treatment is indicated in patients with Grade IV VUR especially in younger patients or those with unilateral disease. Of Urine cultures are performed 3 monthly to exclude the patients with Grade V VUR only infants are trialed breakthrough infection. Annual radiological on a medical approach before surgery is indicated, in investigations are likewise indicated. Good perineal older patients surgery is the only option. hygiene, and timed and double voiding are also Endoscopic Injection Deflux is a gel that is used in endoscopic injections to treat Vesicoureteral Reflux. It is the material that the surgeon injects around the ureteral opening to create a valve function and stop urine from flowing back up the ureter. Deflux consists of two types of sugarbreakthrough infection results despite prophylaxis, or based molecules called dextranomer and hyaluronic there is non-compliance with the prophylaxis. acid. Both substances are well-known from previous Similarly if the VUR is severe (Grade IV & V), there uses in medicine. Both materials are also are pyelonephritic changes or congenital biocompatible, which means that they do not cause abnormalities. Other reasons necessitating surgical significant reactions within the body. In fact, intervention are failure of renal growth, formation of hyaluronic acid is produced and found naturally within the body. important aspects of medical treatment. Bladder dysfunction is treated with the administration of anticholinergics. Surgical Management A surgical approach is necessary in cases where a

Nitrofurantoin{57 mg/kg/24hrs} Nalidixic acid Bactrim Trimethoprim Cephalosporins

new scars, renal deterioration and VUR in girls approaching puberty. There are three types of surgical procedure available for the treatment of VUR: endoscopic (STING/HIT procedures); laparoscopic; and open procedures (Cohen procedure, Leadbetter-Politano procedure).

causing hydronephrosis, and possibly pyonephrosis, kidney failure and sepsis. A person should go straight to an emergency department as soon as possible if unable to urinate when having a painfully full bladder. Causes In the bladder

Urinary retention Urinary retention, also known as ischuria, is a lack of ability to urinate. It is a common complication of benign prostatic hyperplasia (BPH), although it can also be caused by nerve dysfunction, constipation, infection, or medications (including anticholinergics, antidepressants, COX-2 inhibitors, amphetamines and opiates). Diagnosis and/or treatment may require use of a catheter or prostatic stent.

Detrusor sphincter dyssynergia Neurogenic bladder (commonly pelvic splanchic nerve damage, cauda equina syndrome, descending cortical fibers lesion, pontine micturation or storage center lesions, demyelinating diseases or Parkinson's disease)

Iatrogenic (doctor-caused) scarring of the bladder neck (commonly from removal of indwelling catheters or cystoscopy operations)

Damage to the bladder

In the prostate Signs and symptoms Urinary retention is characterised by poor urinary stream with intermittent flow, straining, a sense of incomplete voiding and hesitancy (a delay between trying to urinate and the flow actually beginning). As the bladder remains full, it may lead to incontinence, nocturia (need to urinate at night) and high frequency. Acute retention causing complete anuria is a medical emergency, as the bladder may distend (stretch) to enormous sizes and possibly tear if not dealt with quickly. If the bladder distends enough it will begin to become painful. The increase in pressure in the bladder can also prevent urine from entering the ureters or even cause urine to pass back up the ureters and get into the kidneys,

Benign prostatic hyperplasia Prostate cancer and other pelvic malignancies Prostatitis

Penile urethra

Congenital urethral valves Phimosis or pinhole meatus Circumcision Obstruction in the urethra, for example a metastasis or a precipitated pseudogout crystal in the urine

STD lesions (gonorrhoea causes numerous strictures, leading to a "rosary bead"

appearance, whereas chlamydia usually causes a single stricture) Other

chronic retention, ultrasound of the bladder may show massive increase in bladder capacity (normal capacity being 400-600 ml). Determination of the serum prostate-specific

Paruresis ("shy bladder syndrome")- in extreme cases, urinary retention can result

antigen (PSA) may aid in diagnosing or ruling out prostate cancer, though this is also raised in BPH and prostatitis. A TRUS biopsy of the prostate (trans-rectal ultra-sound guided) can distinguish between these prostate conditions. Serum urea andcreatinine determinations may be necessary to rule out backflow kidney damage. Cystoscopy may be needed to explore the urinary passage and rule out blockages. In acute cases of urinary retention where associated symptoms in the lumbar spine are present such as pain, numbness (saddle anesthesia), parasthesias, decreased anal sphincter tone, or altered deep tendon reflexes, an MRI of the lumbar spine should be considered to further assess Cauda Equina Syndrome. Complications Urinary retention often occurs without warning. It is basically the inability to pass urine. In some people, the disorder starts gradually but in others it may appear suddenly. Acute urinary retention is a medical emergency and requires prompt treatment. The pain can be excruciating when urine is not able to flow out. Moreover one can develop severe sweating, chest pain, anxiety and high blood pressure. Other patients may develop a shock like condition and may be require an admission to the hospital. It is not unusual for an individual to develop a heart attack after suffering acute urinary retention. Other more serious complications of untreated urinary retention

Consumption of some psychoactive substances, mainly stimulants, such as MDMA and amphetamine.

Use of NSAIDs or drugs with anticholinergic properties.

Stones or metastases can theoretically appear anywhere along the urinary tract, but vary in frequency depending on anatomy

Muscarinic antagonist such as Atropine and Scopolamine

Paruresis, inability to urinate in the presence of others (such as in a public restroom), may also be classified as a type of urinary retention, although it is psychological rather than biological. Diagnosis As seen on axial CT Urine flow tests may aid in establishing the type of micturition (urination) abnormality. Common findings, determined by ultrasound of the bladder, include a slow rate of flow, intermittent flow, and a large amount of urine retained in the bladder after urination. A normal test result should be 20-25 mL/sec peak flow rate. A post-void residual urine greater than 50 ml is a significant amount of urine and increases the potential for recurring urinary tract infections. In adults older than 60 years, 50-100 ml of residual urine may remain after each voiding because of the decreased contractility of the detrusor muscle. In

include bladder damage and chronic kidney failure.


a Foley catheter that is emplaced with a small inflatable bulb that holds the catheter in place. Intermittent catheterization can be done by a health care professional (nurse/ doctor) or by the patient himself /herself (clean intermittent self catheterization). Intermittent catheterization performed at the hospital will be a sterile technique

Urinary retention is a disorder which is

treated in a hospital and the quicker one seeks treatment, the fewer the complications. In the longer term, obstruction of the urinary tract may cause:

Bladder stones Atrophy of the detrusor muscle (atonic bladder is an extreme form)

done by nurses /doctores, while patients can be taught to use a self catheterization

technique in one

simple demonstration, and that reduces the rate of infection from longer term Foley catheters. Self catheterization requires doing the procedure every 3 or 4 hours 4-6 times a day. The chronic form of urinary retention may require some type ofsurgical procedure. While both procedures are relatively safe, complications can occur. In acute urinary retention, the treatment requires urgent placement of a urinary catheter (tube) into the urethra and into the bladder. These catheters are usually inserted by health care professionals. However, if the procedure is not accomplished in a sterile fashion, it can introduce bacteria into the bladder. This can result in an infection of the entire urinary tract. Therefore, sterile technique is a must when inserting a foley catheter. Careful washing of hands, meatus, and reusable catheters are also necessary with clean self catheterization techniques.

Hydronephrosis (congestion of the kidneys) Hypertrophy of the detrusor muscle (the muscle which squeezes the bladder to empty it during urination)

Diverticula (the formation of pouches) in the bladder wall (which can lead to stones and infection)

Treatment In acute urinary retention, urinary catheterization, placement of a prostatic stent or suprapubic cystostomy relieves the retention. In the longer term, treatment depends on the cause. BPH may respond to alpha blocker and 5-alpha-reductase inhibitor therapy, or surgically with prostatectomy or transurethral resection of the prostate (TURP). Older patients with ongoing problems may require continued intermittent self catheterization.

5-alpha-reductase inhibitor increase

the chance of normal urination following catheter removal.


Sometimes the permanent urinary catheter may cause discomfort and pain which often lasts for several days. The urinary catheter must be placed into the bladder and not near the prostate gland.

Complications The acute urinary retention is treated by placement of a urinary catheter (small thin flexible tube) into the bladder. This can be either an intermittent catheter or

Placement of the catheter near the prostate can lead to bleeding and significant irritation. In most patients with benign prostate hyperplasia (BPH), a procedure

known as transurethral resection of the prostate (TURP) is performed to relieve bladder obstruction. The surgery is done with a small lighted instrument which is inserted into the urethra under anesthesia. The surgeon can core out the enlarged prostate and relieve obstruction. However, the procedure does have risks. There are risks of anesthesia which may include allergy to medications or low blood pressure which results from spinal anesthesia.

certain medications (blood pressure pills, anti histamines,antiparkinson medications), after spinal anaesthesia or stroke. In young males, the most common cause of urinary retention is infection of the prostate (known as "acute prostatitis"). The infection is acquired during sexual intercourse and presents with low back pain, penile discharge, low grade fever and an inability to pass urine. The exact numbers of individuals with acute prostatitis is unknown because many do not always seek treatment. In the USA, at least 1-3 percent of males under the age of 40 develop urinary difficulty as a result of acute prostatitis. Most physicians and other health care professionals are aware of these disorders. Worldwide, both BPH and acute prostatitis have been found to occur in males of all races, color and ethnic backgrounds. Cancers of the urinary tract can cause urinary obstruction but the process is more gradual. Bladder cancer, prostate or ureters can gradually obstruct urine output. Cancers often present with blood in the urine, weight loss, lower back pain or gradual distension in the flanks.

The surgical complications from TURP include a bladder infection, bleeding from the prostate, scar formation, inability to hold urine and inability to have an erection. The majority of these complications are short lived and most individuals recover fully within 6 12 months.

Some people with BPH are treated with

medications like finasteride or dutasteride to decrease prostate enlargement. The drugs only work for mild cases of BPH but also have mild side effects. Some of the medications decrease libido and may cause dizziness, fatigue and lightheadedness. Unfortunately, medications only work in less than 5 percent of individuals with BPH. Epidemiology Urinary retention is a common disorder in elderly males. The most common cause of urinary retention is BPH. This disorder starts around age 50 and symptoms may appear after 1015 years. BPH is a progressive disorder and narrows the neck of the bladder leading to urinary retention. By the age of 70, almost 10 percent of males have some degree of BPH and 33% have it by the eighth decade of life. While BPH rarely causes sudden urinary retention, the condition can become acute in the presence of

Glomerulonephritis, also known as glomerular nephritis, abbreviated GN, is a renal disease (usually of both kidneys) characterized byinflammation of the glomeruli, or small blood vessels in the kidneys.

It may present with

isolated hematuria and/or proteinuria (blood or protein in theurine); or as a nephrotic syndrome, a nephritic syndrome, acute renal failure, or chronic renal failure. They are categorized into several different pathological patterns, which are broadly grouped into non-proliferative or proliferative types. Diagnosing the pattern of GN is important because the outcome and treatment differs in different types. Primary causes are intrinsic to the kidney. Secondary causes are associated with certain infections (bacterial, viral or

parasitic pathogens), drugs, systemic disorders (SLE, vasculitis), or diabetes. Thin Basement Membrane Disease Thin basement membrane disease is an autosomal dominant inherited disease characterized by thin glomerular basement membranes on electron microscopy. It is a benign condition that causes persistent microscopic hematuria. This also may cause proteinuria which is usually mild and overall prognosis is excellent. Non Proliferative This is characterised by absence of increase in the number of cells (lack of hypercellularity) in the glomeruli. They usually cause nephrotic syndrome. This includes the following types: Minimal change GN (also known as Minimal Change Disease) Main article: Minimal change disease This form of GN causes 78.4% of nephrotic syndrome in children, but only 20% in adults. As the name indicates, there are no changes visible on simple light microscopy, but on electron microscopy there is fusion of podocytes (supportive cells in the glomerulus). Immunohistochemistry staining is negative. Treatment consists of supportive care for the massive fluid accumulation in the patients body (= oedema) and as well as steroids to halt the disease process (typically prednisone 1 mg/kg). Over 90% of children respond well to steroids, being essentially cured after 3 months of treatment. Adults have a lower response rate (80%). Failure to respond to steroids ('steroid resistant') or return of the disease when steroids are stopped ('steroid dependent') may require cytotoxic therapy (such as cyclosporin), which is associated with many side-effects. Focal Segmental Glomerulosclerosis (FSGS) Main article: Focal segmental glomerulosclerosis FSGS may be primary or secondary to reflux nephropathy, Alport syndrome, heroin abuse or HIV.

FSGS presents as a nephrotic syndrome with varying degrees of impaired renal function (seen as a rising serum creatinine, hypertension). As the name suggests, only certain foci of glomeruli within the kidney are affected, and then only a segment of an individual glomerulus. The pathological lesion is sclerosis (fibrosis) within the glomerulus and hyalinisation of the feeding arterioles, but no increase in the number of cells (hence non-proliferative). The hyaline is an amorphous material, pink, homogeneous, resulting from combination of plasma proteins, increased mesangial matrix and collagen. Staining for antibodies and complement is essentially negative. Steroids are often tried but not shown effective. 50% of people with FSGS continue to have progressive deterioration of kidney function, ending in renal failure. Membranous glomerulonephritis Main article: Membranous glomerulonephritis Membranous glomerulonephritis (MGN), a relatively common type of glomerulonephritis in adults, frequently produces a mixed nephrotic and nephritic picture. Its cause is usually unknown, but may be associated with cancers of the lung and bowel, infection such as hepatitis and malaria, drugs including penicillamine, and connective tissue diseases such as systemic lupus erythematosus. Individuals with cerebral shunts are at risk of developing shunt nephritis, which frequently produces MGN. Microscopically, MGN is characterized by a thickened glomerular basement membrane without a hypercellular glomerulus. Immunofluorescence demonstrates diffuse granular uptake of IgG. The basement membrane may completely surround the granular deposits, forming a "spike and dome" pattern. Tubules also display the symptoms of a typical Type III hypersensitivity reaction, which causes the endothelial cells to proliferate, which can be seen under a light microscope with a PAS stain.

Prognosis follows the rule of thirds: one-third remain with MGN indefinitely, one-third remit, and one-third progress to end-stage renal failure. As the glomerulonephritis progresses, the tubules of the kidney become infected, leading to atrophy and hyalinisation. The kidney appears to shrink. Treatment with corticosteroids is attempted if the disease progresses. In extremely rare cases, the disease has been known to run in families, usually passed down through the females. This condition, similarly, is called Familial Membranous Glomerulonephritis. There have only been about nine documented cases in the world. Proliferative This type is characterised by increased number of cells in the glomerulus (hypercellular). Usually present as a nephritic syndrome and usually progress to endstage renal failure (ESRF) over weeks to years (depending on type). IgA nephropathy (Berger's disease) Main article: IgA nephropathy

Presentation 1. Recurrent gross or microscopic hematuria

gross hematuria occurs post-infection of the respiratory (more common), gastrointestinal, or urinary tract

1. 2.

mild proteinuria occasionally nephrotic syndrome (although it usually has a nephritic presentation)

3. Causes

rarely, presents with crescentic Rapidly progressive GN

The disease can be primary or secondary to liver and intestinal diseases. It also overlaps with Henoch-Schonlein purpura, a systemic renal disease in children in which similar IgA deposits occur. Pathology plasma polymeric IgA is increased in IgA Nephropathy, and circulating IgA-containing immune complexes can be found in the blood of some patients. Plasma IgA is usually monomeric and polymeric plasma IgA is broken down in the liver. IgA is normally found in mucus. There are two forms of IgA and only IgA1 causes nephrogenicity. Histology Characteristic finding: 1. 2. 3. IgA deposition in the mesangium seen by immunofloresence Electron dense deposits in electron microscopy Absence of early complement components May find: 1. normal glomeruli

General Information IgA nephropathy is the most common type of glomerulonephritis in adults worldwide. It usually presents as macroscopic haematuria (visibly bloody urine). It occasionally presents as a nephrotic syndrome. It often affects young males within days (24-48hrs) after an upper respiratory tract or gastrointestinal infection. Microscopic examination of biopsy specimens shows increased number of mesangial cells with increased matrix (the 'cement' that holds everything together). Immuno-staining is positive for immunoglobulin A deposits within the matrix. Prognosis is variable, 20% progress to ESRF. ACE inhibitors are the mainstay of treatment. Summary This is a form of GN characterised by IgA deposits in the mesangial regions and immunocytochemistry is required for definitive diagnosis of the disease.

2. 3. 4. 5.

mesangioproliferative GN focal proliferative GN (healing may cause focal segmental sclerosis) overt cresentic glomerulonephritis leukocytes in glomerular capillaries

Type 2 (also known as Dense Deposit Disease) Alternative Complement activation only. C3 Nephritic Factor stabilizes C3 convertase, leading to Hypocomplementemia. Unlike Type 1, no IgG is detected.

Post-infectious Post-infectious glomerulonephritis can occur after essentially any infection, but classically occurs after infection with Streptococcus pyogenes. It typically occurs 1014 days after a skin orpharyngeal infection with this bacterium. Patients present with signs and symptoms of glomerulonephritis. Diagnosis is made based on these findings in an individual with a history of recent streptococcal infection. Streptococcal titers in the blood (antistreptolysin O titers) may support the diagnosis. Light microscopy demonstrates diffuse endocapillary hypercellularity due to proliferation of endothelial and mesangial cells, as well as an influx of neutrophils and monocytes. The Bowman spaceis compressed, in some cases to the extent that this produces a crescent formation characteristic of crescentic glomerulonephritis. Biopsy is seldom done as the disease usually regresses without complications. Treatment is supportive, and the disease generally resolves in 24 weeks. Membranoproliferative/mesangiocapillary GN This may be primary, or secondary to SLE, viral hepatitis, or hypocomplementemia. One sees 'hypercellular and hyperlobular' glomeruli due to proliferation of both cells and the matrix within the mesangium. Usually presents with a combined nephritic-nephrotic picture, with inevitable progression to end stage renal failure. The primary form consists of two types:

In both types, the basement membrane may develop a 'tram-track' appearance, due to duplication and splitting. Rapidly progressive glomerulonephritis Rapidly progressive glomerulonephritis (Crescentic GN) has a poor prognosis, with rapid progression to kidney failure over weeks. Steroid therapy is sometimes used.

Any of the above types of GN can be

rapidly progressive. Additionally two further causes present as solely RPGN.

One is Goodpasture syndrome, an autoimmune disease whereby antibodies are directed against basal membrane antigens found in the kidney and lungs. As well as kidney failure, patients have hemoptysis (cough up blood). High dose immunosuppression is required (intravenousmethylprednisolone) and cyclophosphamide, plus plasmapheresis. Immunohistochemistry staining of tissue specimens shows linear IgG deposits.

The second cause is vasculitic disorders such as Wegener granulomatosis and polyarteritis. There is a lack of immune deposits on staining, but blood tests are positive for ANCA antibody.

Histopathology: The majority of glomeruli present "crescents". Formation of crescents is initiated by passage of fibrin into the Bowman space as a result of increased permeability of glomerular basement membrane. Fibrin stimulates the proliferation of endothelial cells of Bowman capsule, and an influx of monocytes. Rapid growing and fibrosis of crescents compresses the capillary loops and decreases the Bowman

Type 1 (Classical and Alternative Complement activation)

space, which leads to renal failure within weeks or months. Hydronephrosis Hydronephrosis - literally "water inside the kidney" refers to distension and dilation of the renal pelvis and calyces, usually caused by obstruction of the free flow of urine from the kidney. Untreated, it leads to progressive atrophy of the kidney.

affected kidney. Physical examination may detect a palpable abdominal or flank mass caused by the enlarged kidney. Causes Hydronephrosis is the result of several abnormal pathophysiological occurrences. Structural abnormalities of the junctions between the kidney, ureter, and bladder that lead to hydronephrosis can occur during fetal development. Some of these congenital defects have been identified as inherited

In cases

of hydroureteronephrosis, there is distention of both the ureter and the renal pelvis and calices. Signs and symptoms

conditions, however the benefits of linking genetic The signs and symptoms of hydronephrosis depend upon whether the obstruction is acute or chronic, partial or complete, unilateral or bilateral. Hydronephrosis that occurs 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."

testing to early diagnosis have not been determined.


Other structural abnormalities could be

caused by injury, surgery, or radiation therapy. Compression of one or both ureters can also be caused by other developmental defects not completely occurring during the fetal stage such as an abnormally placed vein, artery, or tumor. Bilateral compression of the ureters can occur during pregnancy due to enlargement of the uterus. Changes in hormone levels during this time may also affect the muscle contractions of the bladder, further complicating this condition. Sources of obstruction that can arise from other various causes include kidney stones, blood clots, or retroperitoneal fibrosis.

Conversely, hydronephrosis that

develops gradually will generally cause no pain or attacks of a dull discomfort. 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.

The obstruction may be either partial or complete and can occur anywhere from the urethral meatus to the calyces of the renal pelvis. Hydronephrosis can also result from the reverse flow of urine from the bladder back into the kidneys. This reflux can be caused by some of the factors listed above as well as compression of the bladder outlet

Blood tests may show impaired kidney function (elevated urea or creatinine) or electrolyte imbalances such as hyponatremia or hyperchloremic metabolic acidosis. Urinalysis may indicate an elevated pH due to the secondary destruction of nephrons within the

into the urethra by prostatic enlargement or impaction of feces in the colon, as well as abnormal contractions of bladder muscles resulting from neurological dysfunction or other muscular disorders. Pathophysiology Obstruction that occurs anywhere along the upper urinary tract will lead to increased pressure within the structures of the kidney due to the inability to pass urine from the kidney to the bladder. Common causes of upper tract obstruction include obstructing stones and ureteropelvic junction (UPJ) obstruction caused by intrinsic narrowing of the ureters or an overlying vessel. Obstruction occurring in the lower urinary tract can also cause this increased pressure through the reflux of urine into the kidney. Common causes include bladder dysfunction (such as neurogenic bladder) and urethral obstruction (such as posterior urethral valves in male infants) or compression (such as from prostatic hypertrophy in older male adults). Anything that causes obstruction leads to increased pressure being transmitted to the delicate tissues that make up the filtration system within the kidneys, which could eventually result in infection, stone formation, or loss of function. Additional complications arising from obstruction of the lower urinary tract include the stagnation of urine flow which can also lead to infection in the bladder. Obstruction may be a result of a tumour in the pelvis compressing the ureters or urethra, for example in patients with advanced cervical cancer (stage IIIA to IVB). Diagnosis

Prenatal diagnosis is possible,


and in fact, most

cases in pediatric patients are incidentally detected by routine screening ultrasounds obtained during pregnancy.

However, approximately half of all

prenatally-identified hydronephrosis is transient, and resolves by the time the infant is born, and in another 15%, the hydronephrosis persists but is not associated with urinary tract obstruction (so-called non-refluxing, non-obstructive hydronephrosis). For these children, regression of the hydronephrosis occurs spontaneously, usually by age 3. However, in the remaining 35% of cases of prenatal hydronephrosis, a pathological condition can be identified postnatally.

Diagnostic workup depends on the age of the patient, as well as whether the hydronephrosis was detected incidentally or prenatally or is associated with other symptoms. Blood tests (such measurement of creatinine) are typically indicated, though they must be interpreted cautiously. Even in cases of severe unilateral hydronephrosis, the overall kidney function may remain normal since the unaffected kidney will compensate for the obstructed kidney. Urinalysis is usually performed to determine the presence of blood (which is typical for kidney stones) or signs of infection (such as a positive leukocyte esterase or nitrite). Impaired concentrating ability or elevated urine pH (distal renal tubular acidosis) are also commonly found due to tubular stress and injury. Imaging studies - such as an intravenous urogram (IVU), ultrasound, CT or MRI - are also important investigations in determining the presence and/ or cause of hydronephrosis. Whilst ultrasound allows for

visualisation of the ureters and kidneys (and determine the presence of hydronephrosis and / or hydroureter), an IVU is useful for assessing the anatomical location of the obstruction. Antegrade or retrograde pyelography will show similar findings to an IVU but offer a therapeutic option as well. The choice of imaging depends on the clinical presentation (history, symptoms and examination findings). In the case of renal colic (one sided loin pain usually accompanied by a trace of blood in the urine) the initial investigation is usually a spiral or helical CT scan. This has the advantage of showing whether there is any obstruction of flow of urine causing hydronephrosis as well as demonstrating the function of the other kidney. Many stones are not visible on plain X-ray or IVU but 99% of stones are visible on CT and therefore CT is becoming a common choice of initial investigation. CT is not used however, when there is a reason to avoid radiation exposure, e.g. in pregnancy. For incidentally-detected prenatal hydronephrosis, the first study to obtain is a postnatal renal ultrasound, since as noted, many cases of prenatal hydronephrosis resolve spontaneously. This is generally done within the first few days after birth, although there is some risk that obtaining an imaging study this early may miss some cases of mild hydronephrosis due to the relative oliguria of a newborn. Thus, some experts recommend obtaining a follow up ultrasound at 4-6 weeks to reduce the falsenegative rate of the initial ultrasound.

obstruction is suspected, such as a ureteropelvic junction (UPJ) or ureterovesical junction (UVJ) obstruction, a nuclear imaging study such as a MAG3 scan is warranted. Prognosis The prognosis of hydronephrosis is extremely variable, and depends on the condition leading to hydronephrosis, whether one (unilateral) or both (bilateral) kidneys are affected, the pre-existing kidney function, the duration of hydronephrosis (acute or chronic), and whether hydronephrosis occurred in developing or mature kidneys. For example, unilateral hydronephrosis caused by an obstructing stone will likely resolve when the stone passes, and the likelihood of recovery is excellent. Alternately, severe bilateral prenatal hydronephrosis (such as occurs with posterior urethral valves) will likely carry a poor long-term prognosis, because obstruction while the kidneys are developing causes permanent kidney damage even if the obstruction is relieved postnatally. Treatment Treatment of hydronephrosis focuses upon the removal of the obstruction and drainage of the urine that has accumulated behind the obstruction. Therefore, the specific treatment depends upon where the obstruction lies, and whether it is acute or chronic. Acute obstruction of the upper urinary tract is usually treated by the insertion of a nephrostomy tube. Chronic upper urinary tract obstruction is treated by the insertion of a ureteric stent or apyeloplasty.

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cystourethrogram (VCUG) is also typically obtained to exclude the possibility of vesicoureteral reflux or anatomical abnormalities such as posterior urethral valves. Finally, if hydronephrosis is significant and