URO NEPHROLOGY

PATHOLOGIES

URINE NORMAL RESULTS Urinalysis requires a fresh sample in a clean container done within 2 hours of collection. If is going to be delayed, needs refrigeration, avoiding the decomposition of the sample

Normal urine is a clear straw-colored liquid, with a slight odor. It is affected by diet, concentration, Ds, drugs, and Physical activity.

It contains some crystals, a small number of cells from the tissues that line the bladder, and transparent (hyaline) casts.

Normal urine does not contain sugars, yeast cells, protein, ketones, bacteria, or parasitic organisms.

pH The pH of normal urine is 4.5–8.0. average is 6.0

Acidic urine is associated with xanthine, cystine, uric acid, calcium oxalate stones

Alkaline urine is associated with calcium carbonate, calcium phosphate, magnesium phosphate stones.

Increased Ph ↑ than 7 (alkaline) Decrease Ph ↓ 7 (more acid) Resp/Met alkalosis Resp/Metabolic acidosisVegan diet Diabetes MellitusVomiting StarvationDiuretics DiarrheaUTI Diet high in meat

Abnormal results:

CLOUDINESS: Urine may be cloudy because it contains red or white blood cells, bacteria, fat, mucus, or pus from a bladder or kidney infection.

ODOR The aromatic odor of normal urine is caused by presence of volatile acid.Foul-smelling urine is a common symptom of urinary-tract infection. Fruity odor/sweet smell of acetone: DM, starvation, dehydration, ketones.Some foods (asparagus), and certain antibiotics can affect the urine odor

BLOOD AND TISSUE CELLS Red blood cells in the urine can be due: vigorous exercise or exposure to toxic chemicals.

Bloody urine can also be a sign of bleeding in the G/U tract as a result of systemic bleeding disorders, kidney diseases, bacterial infections, obstructions in the urinary tract, traumatic injuries, and tumors.

A high number of white blood cells in the urine is a symptom of UTI

A large number of cells from tissue lining (epithelial cells) indicate damage to the small tubes that carry material into and out of the kidneys.

SPECIFIC GRAVITY (N-1005/1035)Reflects the concentrating capability of kidneys, and hydration status of patient

Low specific gravity (below 1.005), more dilute the urine: associated with diabetes insipidus, ATN, pyelonephritis, AGN, overhydration, renal failure (decrease water reabsorption)

High specific gravity (above 1.035), more concentrated the urine: patients who are in shock, suffer from nephrotic syndrome, acute GN, CHF, water restriction/dehydration (kidneys reabsorbing water, urine is concentrated), excessive sweating, diarrhea, fever.

PROTEIN Proteinuria can be a symptom of kidney stones, inflammation of the kidneys, degenerative kidney disease, or multiple tumors. Findings of more than 1+ protein with SG under 1015 abnormal protein excretion

CRYSTALS Some appear in normal urine, such as calcium oxalate or uric acid crystal A large number of calcium oxalate crystals, is a sign of abnormally high levels of calcium in the blood. Other crystals, including tyrosine, leucine, and cholesterol, are abnormal.

NITRITES:They are found when nitrate reducing bacteria are present in large numbers. A negative test, does not rule a UTI.

BLOOD AND TISSUE CELLS

Red blood cells in the urine can be due: vigorous exercise or exposure to toxic chemicals.

Presence of more than 5 RBC/HPF in a bloody urine can also be a sign of bleeding in the G/U tract as a result of systemic bleeding disorders, kidney diseases, bacterial infections, obstructions in the urinary tract, traumatic injuries, and tumors.

A high number of white blood cells in urine is a symptom of kidney/UTI disease

A large number of cells from tissue lining (epithelial cells) indicate damage to the small tubes that carry material into and out of the kidneys.

CASTS

Formed when protein and other materials settle in the kidney tubules and collecting ducts. Casts are dislodged by normal urine flow. A large number of white blood or red blood cast cells in a urine specimen is a sign of kidney disease.

LEUKOCYTES ESTERASE: Produced by the granulocytic blood cells during an infection

Presence of 5 or more leukocytes (WBC) per high power field may indicate UTI, interstitial cystitis, calculous disease, and glomerulonephritis.

SUGARS A high level of glucose and other sugars in the urine (glycosuria) is often a symptom of diabetes mellitus. Glycosuria can also be caused by advanced kidney disease, impaired tubular reabsorption, shock, cancer of the pancreas, Cushing.

KETONES The presence of abnormally high numbers of ketones in the urine (ketonuria) usually results from uncontrolled diabetes mellitus. Ketonuria can also be caused by prolonged starvation, or decreased CH intake, Etho, fever

UROBILINOGEN Finding urobilinogen in the urine may be a symptom of liver disease caused by the formation of fibrous tissue, medications that damage the liver, or obstructive jaundice.

BUN: Blood Urea Nitrogen, is a general indicator of renal function. Is influenced by diet, catabolic state of patient

CREATININE: Is a more sensitive indicator of renal function than BUN. Is influenced by muscle mass of patient

Urine Color Range from pale yellow (diluted) to amber yellow (concentrated), due to the pigment urochrome. The color indicates the concentration of urine and varies with the specific gravity.

Abnormal Urine Colors

Red Color: Hematuria/Myoglobinuria: Brownish red color. muscle damage, vigorous exercice

Dark Red Urine: bleeding from kidneys

Bright Red Urine: bleeding in lower urinary tract

Pink diaper syndrome: pinkish color on diaper 2ary to urate crystals in neonates

Foods: Berries, beets, food coloring

Drugs: Dilantin, phenothiazines,

Orange: Pyridium, Rifampin, Urate Crystals

Green: Food coloring, Pseudomonas infection

Brown/Black: Hemoglobin, Bilirubin, Flagyl, Nitrofurantoin

Dark Yellow: Bile pigments (urobilinogen or bilirubin), ↑ Carotene in diet

Blue: Methilene blue, Defect in the tryotophan absorption

Milky/Cloudy Color: Pus/Bacteria in urine. Also large amount of fat foods

MALE GENITALIA:

Testes descends into well-formed, pigmented and rugated scrotum, or are in the inguinal canal Urethral normal opening: At center of shaft. Hypospadia / Epyspadia Phymosis Hydrocele Paraphymosis Neonatal cryptorchidism. Balanitis Testicular Torsion (swollen, bluish)

In the first week, the urine can assume a red brick color represented by urates, staining the diapers as blood. They disappear in water, being a differential diagnosis with hematuria

Phimosis/Paraphimosis

Phimosis: Inability to retract the prepuce (foreskin). Normal in children under 3 years.

In older males, phimosis may be due to the inflammation at the tip of the foreskin

Paraphimosis: Inability to reduce the foreskin due to venous congestion of the foreskin.

Can progress to arterial compromise and gangrene

S/S: Phimosis can cause urinary retention due to pain or obstruction of the urethra

TX:

Phimosis: Steroid cream applied to foreskin to loosen the phimotic ring.

In chronic phimosis- Circumcision

Paraphimosis: Lubrication and compression of the foreskin and glans. In refractory cases,

a vertical incision of the ligating band is done.

PARAPHYMOSIS

BALANOPOSTHITIS-BALANITIS (balanitis, of the glans; posthitis, of the prepuce)

Balanitis is an inflammation of the foreskin and head of the penis.

Balanitis is usually caused by poor hygiene in uncircumcised men.

The inflammation can be due to a bacterial, fungal, or viral infection, caustic soaps, or failure to properly rinse soap off while bathing.

Symptoms:    redness of foreskin or penis, rashes on the head of the penis, foul-smelling discharge, painful penis and foreskin

Treatment:    Tx depends on the cause of the balanitis. Infectious balanitis may be treated with ATB or ointments, good hygiene. Circumcision-severe case

Complications:    Chronic inflammation or infection can:

Scar and narrow the opening of the penis (urethral stricture) Painful retraction of the foreskin to expose the tip of the penis( phimosis) Difficulty to reposition the foreskin to cover the head of the penis (PP); swelling can affect the blood supply to the tip of the penis

Prevention: Good hygiene

BALANITIS

HYDROCELE

Accumulation of peritoneal fluid in the tunica vaginalis of scrotum, due to obliteration of processus vaginalis

At about 28 weeks of gestation, the testicles descend from the developing baby's abdominal cavity into the scrotum. A sac (processus vaginalis) accompanies each testicle, allowing fluid to surround them. In most cases, the sac closes and the fluid is absorbed. If the fluid remains after the sac closes, the condition is known as a noncommunicating hydrocele. Because the sac is closed, fluid can't flow back into the abdomen. Usually the fluid gets absorbed within a year.

In some cases, however, the sac remains open. With this condition, known as communicating hydrocele, the sac can change size or, if the scrotal sac is compressed, fluid can flow back into the abdomen.

S/S: Non tender swelling of scrotum, round and smooth Distal testicular shadow can be seen by transillumination

R/O: Hernia. Hydroceles transilluminates and does not reduce. Hernia are reducible

TX: Spontaneous regression in 90% of cases. Surgery- Hydrocelectomy, only after the first year of life, particularly if is communicating hydrocele or inguinal hernia is present at the same time

HYDROCELE

HYPOSPADIASIs when the urethral meatus is located below and proximal to its normal position

The ventral foreskin is lacking while the dorsal portion has a hood aspect

The ventral urethral meatus- in most cases opens on or adjacent to the glans penis, but the opening can be on the shaft or in the perineum.

Chordee- is a ventral curvature of the penis shaft, most apparent on erection.

Patients with hypospadia should not be circumcised, because the foreskin is necessary for later repair.

The ideal age is between 18 and 24 months.

HYPOSPADIAS

HYPOSPADIAS

Epispadias.

URINARY TRACT INFECTIONIs an infection from the bladder until the kidneys, occurring when bacteria enter the opening of the urethra and multiply in the urinary tract, where can lead to a significant damage

Types:

Urethritis: Infection of the urethra

Cystitis: Bladder infection is by far the most common UTI.

Pyelonephritis: Inf. of renal parenchyma and renal pelvis, due to

ascending infection from bladder, is due to G-

Etiology: Most common: E. coli 75-90%%

Less common: Klebsiella sp, Proteus mirabilis, Enterococcus or other G-

Uncommonly: staph saprophyticus

Epidemiology:

•Females are more prone to have UTI: Shorter urethra Proximity to the anus Soiled diapers/irritation of the perineum Wiping from back to front Sexual intercourse Bladder instrumentation Delayed diaper changing

•Urinary tract abnormalities: Vesico-ureteral reflux Urethral obstruction Neurogenic bladder Urologic anomalies Sexual activity Incomplete voiding Phimosis in uncircumcized boys Urethral instrumentation

S/S:

Babies: Fever, Poor feeding, Irritability, Abdominal pain, Vomiting, FTT, ↑ jaundice

Older children Lower tract infection: CYSTITIS

Urgency, Frequency, Dysuria, suprapubic (Bladder) tenderness, hematuria, nocturia, voiding in small quantities.

Older children Upper tract infection: PYELONEPHRITIS

Chills, N/V, flank/back pain, fever, CVA- tender to percussion, Abdominal tenderness or mass, tachycardia •Examine external genitalia for signs of irritation, pinworms, vaginitis, trauma, or sexual abuse.

LAB:

Urinalysis: Screening Dipstick (Nitrite, Leukocyte esterase) Microscopy

Presence of nitrite most specific, because bacteria convert nitrate to nitrite in bladder, best detected in morning sample. (nitrate splitting bacteria, specially G-)

Leukocytes (leukocyte esterase) dipstick test - Detects esterases released from broken down Leukocytes- pyuria.

Urinalysis-5 and up WBC/HPF (bacteria on Gram stain/high power field

Pyelonephritis: Leukocytosis with left shift. WBC, WBC casts, RBC, pyuria, Urine Culture +

Urine culture:*Midstream urine sample-positive over 100.000 colonies

*Catheterized specimen-positive over 10.000 colonies

*Urine culture via suprapubic tap (sterile)- Positive 1.000 col.

*If positive for UTI, check BUN/Creatinine and BP renal function

*Urine Culture: sterile collection is the Gold Standard for Dx

IMAGING:

*Renal U/S- Noninvasive evaluation of structural defects. It shows kidney size/shape, but it poorly show ureters,provides no information on function. Diagnose urolithiasis, hydronephrosis, hydroureter, and bladder distention replacing the intravenous pyelogram (IVP) in many cases.

*DMSA Renal scan: This study frequently uses technetium Tc 99m dimercaptosuccinic acid (DMSA).Detects tubular damage and scarring and shows the kidney outline, but it does not show the collecting system.

*Voiding Cystourethrogram (VCUG): Shows urethral and bladder anatomy and detects vesico-ureteral reflux (VUR). Girls < 5 years with febrile UTI, or males with 2 separate UTI

After first positive culture UTI: Renal Ultrasound VCUG initially, later nuclear cystogram Renal Scan: DMSA (R/O scarring, even if negative VUR) Mag – 3 (obstruction)

TX:

Lower Tract Infection- Cystitis: Amoxicillin, Fluoroquinolones, Cephalosporines, Nitrofurantoin, TMP/SMX- Septra. PO 5-10 days

Upper Tract Infection: Pyelonephritis: May required hospitalization. Ampicillin/gentamicin, Cefotaxime, Ceftriaxone. IV/IM 10-14 days Amoxicillin, Bactrim, PO

PREVENTION: -Correct wiping-front to back (toilet training) -address poor hygiene -address pinworm irritation-eliminate prolonged baths and bubble bath soaps-address issues of sexual intercourse/trauma/abuse -promote frequent and complete voids-consider circumcision in males with phimosis.-Drink plenty of fluids

Common Antimicrobial Drugs Used in Pediatric Urinary Tract Infections

Drug  Dosage and Interval

Parenteral Therapy  

Ampicillin 100 mg/kg/d 12 h (< 1 week) q 6-8 h (> 1 week)

Ceftriaxone* 25-50 mg/kg/d q 12-24 h

Cefotaxime 150 mg/kg/d q 6-8 h

Gentamicin 5 mg/kg/d q 12 h (< 1 week) 7.5 mg/kg/d q 8 h (> 1 week)

Oral Therapy  

Amoxicillin† 45 mg/kg/d q 8 h

Augmentin 45 mg/kg/d q 12 h

Trimethoprim/ Sulfamethoxazole (Septra )

8 mg/kg/d TMP, 40 mg/kg/d SMX q 12 h 40/200mg/5 ml suspension

Cephalexin (Keflex) 25-50 mg/kg/d q 6 h 125/250 mg suspension/5 ml

Cefixime (Suprax) 8 mg/kg/d or 4mg/kg q 12 h 100 mg suspension/5 ml

* Should not be used in neonates because of potential biliary pseudolithiasis. If cocci are present in urinary sediment, ampicillin should be added until culture and sensitivities are available.

HEMATURIADefine as abnormal number of RBC in urine with more than 3 rbc-hpf.

Origin: Sign/symptom Upper tract Lower tract

Pain CVA/none suprapubic

Dysuria

Blood Clots Absent. Only + Common

with trauma

Cellular cast Common Absent

Proteinuria > 150mg/dl Common Absent

RBC Distorted Normal

CLINICAL AIDS: DISTINGUISHING THE ORIGIN OF GROSS HEMATURIA

Test for: Glomerular ExtraRenal *

Urine color Brown, tea-colored Present

RBC Casts Present Absent

Red cell Morphology Dysmorphic/distorted Normal RBC shape

Urine Stream Bloody More bloody at initial (distal urethral origin) or at

end suggesting trigonitis

Proteinuria >2+ NO

Is divided in:

pre-renal: proteinuria

Renal: presence of RBC cast (GN), proteinuria

Postrenal: absence of RBC cast and proteinuria: UTI, tumors, trauma, stones

Evaluation of Hematuria:

U/A with CX, BUN/Creatinine, Complements, ANA, IgA, Renal Biopsy

Renal/bladder U/S- Whenever gross hematuria

Differential diagnosis of hematuria

IgA nephropathyAcute poststreptococcal glomerulonephritisUrinary tract infectionMembranous glomerulopathySystemic lupus erythematosus ExerciseDrugsStonesGoodpasture diseaseAnaphylactoid purpuraHemolytic-uremic syndrome Membranoproliferative glomerulonephritisCoagulopathiesSickle cell diseaseTraumaTumors

VESICO-URETHERAL REFLUXIs the passage of urine from the bladder to the renal pelvis, secondary to an incompetent valvular mechanism at the uretero-vesical junction

Epidemiology: Family factors- 34% found to have VUR.

Classification: REFLUX:

Grade I- Into a non-dilated distal ureter

Grade II- Into the upper collecting system, with normal calyceal and no dilatation

Grade III- Mildly dilated/tortuous ureter, with mild blunting of calyceal

Grade IV- Grossly dilated ureter, moderate dilatation/tortuosity of ureter.

Grade V- Massive reflux with gross ureteral dilatation and tortuosity of ureter

S/S: UTI sx, HTN, Abdominal mass secondary, Hydronephrosis, FTT, Enuresis

LAB: Voiding cystourethrogram (VCUG)- Gold standard Renal U/S- detects hydronephrosis DMSA renal scan- detects renal scarring DMSA: Dimercaptosuccinic acid

TX:

Incidence of renal scarring, is proportional to the amount of reflux

Most VUR resolves spontaneously grade I-II-III

Grade IV-V unlikely to resolve spontaneously Surgery

Deflux- Dextranomer/ Hyaluronic acid copolimer injection- Resolve 88-67% of Grade I-II-III-IV

ATB prophylaxis (amoxycillin-2 months)Monitor by VCUG every 12-18 months, urine cultures.

GLOMERULONEPHRITIS (APSGN)

Renal Immune complex disease caused by Group A-B hemolytic streptococcus types 12/49 after 1-3

weeks of a skin/throat infection.

characterized by HEMATURIA, PROTEINURIA, HTN, OLIGURIA, EDEMA (sodium/water retention)

Classification:

*Low serum complement level- Systemic diseases

-autoimmune disease: systemic Lupus erythematosus

*

Low serum complement level- Renal diseases

-Acute poststreptococcal GN (APSGN)

-Membranoproliferative GN types 1, 2, 3

*

Normal serum complement level: Renal diseases

-IgA nephropathy

Epidemiology: APSGN occur in all ages. Most frequent in males between 5-15 years.

Rare < 3 years.

1-3 weeks after Strep infection

S/S: Prior throat or skin infection (3-4 weeks)- tonsillitis/impetigo

APSGN: Edema (periorbital-AM, peripheral (feet and/or ankle-PM), Hypertension, Fever, Pallor

Hematuria (Brown coke color), Oliguria, Fatigue, Lethargy, Basal pulmonary crepitations. Seizure (hypertensive encephalopathy- mild ↓ of GFR with serious intravascular expansion and

HTN- edema),

Acute phase resolves about 2 months, abnormalities can persist for over one year

IgA NEPHROPATHY (Berger’s Disease):

Most common glomerular disease worldwide.

IgA deposits in the Mesangial layer

More common in boys, white>black

Hematuria (Berger Ds), mild proteinuria, 10% HTN. Associated with URI

No efficient treatment.

Daily dose of 4 gr of fish oil-fatty acid (Omega-3) with 400 U/day Vit-E: retard renal function loss.

ACE inhibition used in any renal ds where proteinuria and HTN is present

Immunosuppressive tx with prednisone and Azathioprine has shown promise in uncontrolled trials and long term treatment (1-2 y)

ESRD: 5-10% progress to renal failure in children/adolescents, 40% in adulthood

C3 COMPLEMENT is normal

Goodspasture Syndrome:Rapidly, progressive disease, presents with anti-GBM antibodies (Glomerular Basement Membrane) in the glomeruli.

Affects the lungs and kidneys, often with pulmonary hemorrhage.

Presents hemoptysis and nephritic syndrome

Tx: Therapy with steroids, cyclophosphamide and azathioprine alone or in combination with

plasmapheresis to remove circulating immune complexes.

Wegener’s Granulomatosis:Is a systemic vasculitis characterized by nephritis and pulmonary or sinus disease.

Antineutrophil cytoplasmic antibody (ANCA) may be pathogenic in this Glomerulomephritis.

It is a useful marker of disease activity

Rapid progression: pulse methylprednisolone is indicated

Most cases: prednisone plus cyclophosphamide for 1-2 years

LAB:

Throat culture positive for B-hemolytic Streptococcus.

Urine microscopy: crenated RBC’s , RBC cast., WBC, proteinuria

Chemistry: Shows degree of renal failure- Elevation of BUN/ Creatinine Clearance / K

Complement C-3 serum level- LOW

ASO titer, Streptozyme test or Anti-DNase B: positive and elevated titers in patients with APSGN

ESR/CRP: Elevated

ECG: assess hyperkalemia

CXR: Check pulmonary edema and cardiac size.

Renal U/S: to assess size and parenchyma texture

RPGN: Rapidly progressing glomerulonephritis: Anti-GBM/ANCA

Anti-GBM: Goodpasture Syndrome

ANCA: Wegener’s granulomatosis

TX:

*Diet- restricted Sodium, Fluids, Potassium *Loop Diuretics: Furosemide- Lasix.

For volume, BP and K control *Potassium lowering agents: Kayexalate, Insulin *Anti-hypertensive agents: *Vasodilators such as CCB- Nifedipine, lasix

*IV hydralazyne, Na nitroprussiate: for severe hypertension treatment *ATB- Penicillin *Immunosuppressive agents: Prednisone, cyclophosphamide Treat all except APSGN

NEPHROSIS: Is a glomerular disorder characterized by:

Heavy proteinuria, hypoproteinemia, hypercholesterolemia (hyperlipidemia), Anasarca (Edema)

Etiology:

Primary or idiopathic(90%)-Minimal Change Nephrotic Syndrome (85%)

-Focal segmental glomerulosclerosis (10%)

-Mesangial proliferation (5%)

Secondary cases(10%)

-Associated with systemic ds: SLE, Henoch-Shonlein purpura, IgA GN

-Associated with infections/Allergen induced (bee stings, snake bites)

-Drug induced ( Penicillamine, Gold, NSAID’S)

Other:

< 1 year: cong. infection, syphilis, congenital Nephrotic syndrome

>10 years: Membrano-proliferative GN or membranous GN is common

Pathogenesis: Decreased in plasma oncotic pressure secondary to protein loss in urine, LEADS to edema, causing decrease perfusion pressure, and increase of Na+ uptake by kidney.

Prognosis:Minimal change – 80% sustained remission in adolescence

Focal Sclerosis –progress slowly toward end-stage renal disease– dialysis and transplant

S/S: Fatigue, weight gain, facial swelling, periorbital edema, SOB, abd. distention ascites, pitting

edema, pedal edema (cannot put shoes), scrotal swelling, Anasarca, Oliguria, Mild or rare HTN.

LAB: Proteinuria over 4mg/kg/hour or 3,5/4g/day Hyperlipidemia ( ↑ chol, trig, Ldl) Hypoproteinemia or hypoalbuminemia ↑ alfa-2 globulin, ↓ gama globulin Lipiduria with presence of Oval fat bodies, broad waxy casts Protein/Creatinine ratio is > 1.0 (<0.2-0.5mg/dL) Serum albumin, BUN, Creatinine, Urinalysis of 24 hours Complements C3, C4, ANA Renal Biopsy (above 8 years. Etiology-minimal change ds) Microscopic hematuria

TX:

*Diet- restrict salt and fluids, Rest

*Corticoids:

Prednisone 2mg/kg/d PO 4 weeks. Later 1,5 mg/kg/d PO 4 wk.

Proteinuria is normal Taper Prednisone for 3-6 months.

*After 4 weeks of Steroid Tx or 2 relapses in 6 months proteinuria still high Steroid Resistant

renal biopsy cytotoxic drugs (Clorambucil, Cyclophosphamide) or Immunosuppressants such as

Cyclosporine)

*Diuretics: Furosemide (Lasix), Spironolactone

*ATB- PCN prophylaxis

*Pneumovax vaccine

Minimal change disease in a 2-year-old boy with nephrotic syndrome. Eyelid edema in any child should prompt the performance of urinalysis

SCROTAL

SWELLING

Proteinuria- Is the presence of protein in the urine.

Normal less than 150mg/ 24hours

Primary glomerular disorders

Tubular: Tubule damage prevents protein reabsorption:

Ischemic Tubular Injury, Reflux nephropathy, Interstitial nephritis

Glomerular: Damage: results ↑ permeability of glomerular capillary wall

Minimal Change Ds.- mc in school age

Membranoproliferative GN Membranous GN

Messangial proliferative GN Henoch-Shonlein

IgA nephropathy SLE

Non-pathologic: Orthostatic, Febrile, Exercise proteinuria

Secondary disorder: Hereditary: IDDM, Alport

Autoimmune: SLE, Goodpasture’s, Wegener’s

Infectious: Endocarditis, Heroin, Gold, mercury

Neoplastic: Lymphoma, leukemia, Myeloma

Types of proteinuria:

Overload: Because of high levels in blood stream (Bence-Jones protein)

Orthostatic: proteinuria only when erect, none when laying down

Tubular: when is generated by renal tubules

Glomerular: Glomerular leak of protein

S/S: Proteinuria, HTN, Edema, Ascitis, Purpura, Suprapubic/CVA pain

LAB: Urine colormetric dipstick: measure urine protein levels: 1+=30mg/dl 2++=100mg/dl 3+++=300mg/dl 4++++=2000mg/dl 24 hour collection of urine protein excretion BUN, creatinine, total protein, albumin, cholesterol Urine culture ASO titer Creatinine clearance Urine sediment C3, C4, ANA

ENURESIS: Involuntary discharge of urine after the age of expected bladder control, between 5-6 years.

Epidemiology:

Diurnal (small group) more common in females

Nocturnal (majority of cases-Bedwetting) 2-3X more in males

Urinary Control: Between 3-4 years

Primary enuresis: Continence never achieved (never dry) smaller bladder cap.

2 y., less psychological Sx.

Secondary enuresis: Incontinence recurs after a dry period of 3-6M . More in females.

More psychological Sx.

Primary nocturnal enuresis:

20% at 5 years, 10% at 7/8 years, 5% at age 10 years, 1% adults

Primary diurnal enuresis: 1% 7-12 years

Enuresis Impact:Causes significant child emotional distress, and lower self esteemEmbarrassment, Reluctance in participating in overnight activitiesCause the family anger and frustration

Etiology: Low functional bladder capacityHigh urine production during sleep-decrease ADH productionMaturational delays causing Sleep/arousal disturbance Delay in adequate neuromuscular bladder control . MccOther: 30% of encopretics have enuresis Comorbid ADHD, Anxiety, Bipolar

Genetics: 70% of enuretics one parent was enuretic 77% of enuretics both parents were enuretic 15% of enuretics neither parent were enuretic

S/S: Hx. of patient and Family, Vital sign and growth parameters,

Physical Exam. R/O UTI pathologies

Evaluation of Enuresis:Day/night, post void, stream, effort, sign of urge, UTITime of occurrence, times/week, times/night, amount void, longest dry periodStools: frequency of stools, soiling, straining, hard stoolsSleep: condition (cold/damp) available toilet, sleep apnea, daytime sleepinessParental and siblings attitudeFH, PMH, ROSSocial Hx: Stress at home, school, friends

LAB: Urinalysis (glucose), urine culture and microscopic exam R/O: DM, UTI, Diabetes insipidus If there is Hx of UTI: Renal U/S, Voiding Cystourethrogram If suggestive of neurologic dysfunction: Urodynamic studies

TX: Education, Reassurance, Avoid punishment, praise, start chart Decrease fluids at night. Empty bladder before sleeping Desmopressin (DDAVP): ADH- Not to give under 6 years old Decrease overnight urine volume Can be given intranasal. Imipramine: useful, 65-67% of improvement

WILMS TUMOR (Nephroblastoma)

Malignant tumor of the kidney in the pediatric age.

Epidemiology:

5% of all childhood cancer50% occurs under 3 years oldPeak age: 2-4 yearsHigher incidence in Black female childrenAssociated to congenital anomalies

Genetics:

15-20% are hereditary in originFamilial cases are often bilateral and occurring at earlier age

S/S:

Fever, anorexia, hematuria, abdominal pain/distention, anemia, HTN- 60%, due to obstruction of renal art. P.E.: Abd. mass extending from flank toward midline abd. Is unilateral. Associated with aniridia, cryptorchidism, horseshoe kidney

Metastasis to: Lung, liver, bone, brain

LAB: CBC, Lytes, Urine analysis (micro/macro hematuria), liver/kidney profileBiopsy and staging of tumor (prognosis depend on staging and Bx.)

Imaging: U/S of abdomen CT of abdomen Chest X-Ray: R/O chest metastasis

Dx: CT and Bx

TX: Surgery- Nephrectomy, sometimes followed by Chemo and Radiation

Radiation: for stages III/IV or pulmonary metastasis

Chemo: Stages I/II- Vincristine / actinomycin D, every 3 weeks for 6 M Stages III/IV- Vincristine, AMD, doxorubicin for 6-15 months.

STAGING:

Stage I- Tu. restricted to one kidney and completely resected. Renal capsule intact

Stage II- Tumor extends beyond the kidney but is completely excised

Stage III- Residual nonhematogenous tumor is confined to the Abd.

Stage IV- Hematogenous spread to lungs and liver

Stage V- Bilateral disease

Prognosis: Favorable: Stage I/II- more than 90% cured Age at presentation less than 24 months Tumor weight less than 250g Poor: Anaplastic pathology Lymph node involvement Distant metastasis

Displacement of left

renal calyces by Tu.

Testicular Torsion

Testicular torsion occurs when a testicle gets twisted around, cutting off its blood supply.

The patient with this condition usually has severe pain in the testicle.

Due to inadequate fixation of the testes to the scrotum allowing for excessive movement

Venous obstruction edema interrupted arterial flow necrosis

Clinically – Sudden acute testicular pain, scrotal swelling and very tender, absent cremastric reflex

Dark purple color of the scrotum/testicle

Diagnosis: Ultrasound Nuclear medicine scan Treatment: Surgical detorsion

If pain <4-6 hrs, can try manual detorsion

90% survival in testis torsion <6 hrs

Cryptochidism

3-4% full term males (30% of preterm)

10-25% bilateral

Usually in the inguinal canal

Most descend spontaneously by 3 months, 80% by 9-12 months, later it will not descend

Intraabdominal or high up in inguinal canal

Consequences: Infertility, associated hernia, torsion Malignancy risk 1 in 80 in unilateral, 1 in 40 in bilateral

Seminoma, pk 15-45 yrs

If testis not palpable, asses via Ultrasound or laparoscopy

hCG hormone shots for stimulation

Recommend treatment 9-15 months by orchipexy