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Pediatric GU Disorders Month 4 Week 1 T3
Pediatric GU Disorders Month 4 Week 1 T3
34
Medical
Graduate
11/23/2018

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Term
Hematuria
Definition
bloodintheurine.
• Redurinecaneitherbeheme-positiveor heme-negative.
• Heme-positiveurineiscausedbyhemoglobin (protein of blood) or myoglobin (protein of muscle). Heme-negative urine is caused by numerous drugs, dye, and some foods.
• Microscopicevaluationisneededtodetermine hemoglobinuria or myoglobinuria.
Term
Hemoglobinuria
Definition
• The presence of hemoglobin free from red blood cells
• Occurs with rapid disintegration of red blood cells, exceeding the ability of blood protein to bind with hemoglobin
• Hemolytic anemia
Term
Myoglobinuria
Definition
• Skeletal muscle injury
• Can be seen following trauma,
or even after exercise
• If myoglobinuria is present, there is a five-fold increase in serum CK being elevated
• Seen in rhabdomyolysis due to the acute skeletal tissue destruction
Term
Evaluation of child with hematuria
Definition
requires detailed history, PE, and microscopic urinalysis.
• Familyhistory:hereditaryrenaldisease,SLE,sickle cell disease
• Socialhistory:recentexposures,recentillness, meds, menstrual cycles, recent trauma
• PE:vitals(BP),edema,hepatosplenomegaly, abdominal mass, anatomic abnormalities of external genitalia
• Thesefindingshelpdeterminetheurgencyofthe situation and help guide how you will proceed
Term
Hematuria
If child is not acutely ill, and no other worrisome findings on exam
Definition
repeat UA (first AM) on two other occasions within
1–2 weeks.
• If hematuria persists, order urine culture, renal ultrasound, renal panel, CBC. R/O sickle cell if African American.
• Treat based on findings.
Term
Benign familial hematuria
Definition
• It is defined as isolated asymptomatic hematuria without renal abnormalities in multiple family members.
• Presence of persistent microscopic hematuria, often initially seen in childhood. May be intermittent. No treatment.
Term
UTI
Definition
• Defined as bacteria in urine
• Most caused by E.coli, followed by Klebsiella and Proteus, and staph
• Prevalence 3–5% of girls, 1% of boys
- Often peaks during toilet training
- Seen more often in uncircumcised boys


• Urine complaints are common in the primary care setting, and much counseling and education for parents is needed.
• Only treat if culture confirmed UTI and symptoms are present.
• Urine collections in kids can be difficult. Try to get a mid- stream collection whenever possible. (urine hat)
• In infants, sterile collection bags work fairly well.
• However, if diagnosis is uncertain and a greater assurance is needed, a urine cath sample is needed.
• If a collection is obtained at home, the sample must be kept cold until processed.

• Imaging should be done in kids with first UTI before age 5, febrile UTI, recurring UTI, or male with UTI. Image of choice is voiding cystourethrogram (VCUG). This is typically done about 2 weeks following UTI to allow inflammation to resolve.
• Renal ultrasound can also be beneficial to determine size, shape, and renal abnormalities.
Term
Pyelonephritis
Definition
• Bacteriainvolvementofupperurinarytract
• Presentswithabdominalpain,flankpain,fever, lethargy, nausea, vomiting (ill-appearing)
• Diagnosisbyurinalysis,urineculture,PE findings. Renal ultrasound may be helpful if diagnosis is not clear, and will reveal enlarged unilateral kidney
• Mayneedin-patientcareifdehydrated,concern of sepsis, or unable to hold fluids down
• Antibiotictherapydependentonurineculture
Term
Cystitis
Definition
• Bacteriainvolvementofbladder
• Presentswithdysuria,frequency,urgency, sometimes odor, abdominal pain, and incontinence (often seen in older kids); rare fever.
• Diagnosismadebyhistory,PE,urinalysis,and urine culture (imaging not needed unless recurring UTIs)
• Treatpromptlytopreventprogressiontopyelo
• Useurineculturetoguideantibioticselection
Term
Asymptomatic Bacteriuria
Definition
• Defined as positive culture without
symptoms
• Almost exclusive to girls
• Counsel patient and parent about signs/symptoms of UTI and encourage close follow-up if any concerns develop
• Often see this condition in people with long-term catheter use
Term
Proteinuria
Definition
• As many as 10% of routine urine dipstick screenings will be positive for protein in the 8–15-year-old population. It can be challenging to determine whether the etiology of proteinuria is benign or pathological.
• The dipstick detects albuminuria.
• Reported as trace (10-20mg/dL), 1+ (30mg/dL), 2+ (100mg/dL), 3+ (300mg/dL), 4+ (1000+mg/dL)
Term
Ifproteinuriaisfoundondipstickwithabsenceof other findings or concerns
Definition
repeat the dipstick on 2– 3 other occasions (preferably first AM urine).
• Ifpersistingproteinuria,a24-hourcollectionisthe test of choice and the upper limit of normal protein excretion is 150mg/24 hour.
• Inachildwithrepeatnormaldipstickorachildwith a normal 24-hour excretion level, further workup is not indicated.
Term
Orthostatic proteinuria
Definition
s the most common cause of persisting proteinuria in kids.
• This is benign and not associated with an underlying renal abnormality.
• Normal to low levels of protein are excreted in the supine position, but higher levels are excreted in the upright position.
• Cause of this condition is unknown.
Term
Primary Nephrotic Syndrome
Definition
- Minimalchangedisease
• Mostcommon.Glomeruli(capillarynetwork)generally appear normal, or minimal increase in mesangial cells (support cells for glomeruli). Excellent response to corticosteroid therapy.
- Mesangialproliferation
• Leastcommon.Increaseinmesangialcells.About50%of patients respond to corticosteroid therapy.
- Focalglomerulosclerosis
• Mesangialproliferationandsegmentalscarring,leadingto sclerosis. Only 20% of patients respond to corticosteroids and condition is progressive, leading to end-stage renal failure in most patients.

• This disease is more common in males (2:1).
• Generally appears between ages 2–6, although it can be seen in infancy and adulthood.
• Initial episode often follows illness, infections, or allergic reaction.
• Presentation typical of facial and lower extremity edema (often overlooked as an allergic reaction). Edema becomes more progressive and generalized over time. Can lead to ascites, pleural effusions. Abdominal pain, diarrhea and irritability are common. HTN and hematuria are not common early on.
• Different diagnosis: CHF, allergic reaction, protein malnutrition.

Diagnosis
• 3–4+ proteinuria on dipstick (persisting) • Urinary protein exceeds 150mg/24hr
• Serum creatinine is normal to minimally elevated
Term
Primary Nephrotic Syndrome treatment
Definition
Treatment
• Childrenwithgeneralizededema(including ascites, pleural effusion, or otherwise symptomatic) should be hospitalized.
• Diureticsmaybeusedverycautiouslyandunder the direction of a pediatric nephrologist.
• Closemonitoringofvolumestatusismandatory. Complications include volume overload, electrolyte imbalance, HTN, cardiac and renal failure.

Corticosteroid treatment
• If initial episode occurs between ages 1–8, it is likely to be minimal change disorder, and it is generally considered safe to initiate steroids without a biopsy.
• However, if initial episode occurs prior to age 1 or over age 8, or findings of hematuria or HTN, a renal biopsy should be considered.
• Side effects of steroids include weight gain, cushingoid, cataracts, growth failure, HTN.
Corticosteroid treatment
• Initiate treatment of prednisone at a dose of 60mg/day, divided BID or TID for 4–6 weeks. (Should see protein negative urine dips within 10 days.)
• Taper dose to 40mg every other day after 4–6 weeks, and continue gentle taper over two months. (If proteinuria persists beyond 8 weeks, biopsy should be performed to confirm diagnosis.)
• Relapses should be treated with above starting dose until child is dipstick protein negative for three consecutive days, then taper over next 1–2 months.
• Other treatments
- Alternate agents can be used for children who cannot tolerate steroids, are steroid resistant, or who suffer frequent relapses. Both are immune suppressive drugs.
- Cyclophosamide 2–3mg/kg/24 for 8–12 weeks - Side effects include neutropenia, hemorrhagic cystitis, alopecia, sterility, and increased risk of malignancy.
- Cyclosporine 3–6mg/kg/24 for extended periods- Side effects: HTN, nephrotoxicity, hirsutism. Relapse is almost guaranteed if drug is discontinued.
Term
Secondary Nephrotic Syndrome
Definition
Nephrotic syndrome also occurs as a secondary feature of many forms of glomerular disease. Consider secondary nephrotic syndrome in patients greater than 8, HTN, persisting hematuria, renal dysfunction, rash, and/or arthralgia.
• Membranous nephropathy
• Membranoproliferative glomerulonephritis • Post-infectious glomerulonephritis
Term
Glomerulonephritis
Post-Streptococcal glomerulonephritis
Definition
• Sudden onset hematuria, edema, HTN, renal insufficiency.
• Follows a strep infection (group A beta-hemolytic). Sporadic cases. Unclear why some strains lead to disease. Thought again to be immune mediated. Stress importance of compliance when treating strep infections.
• Kidneys appear enlarged on imaging, and glomeruli are enlarged with mesangial cell proliferation.
• Most common between ages 5–12. Symptoms appear 1–2 weeks following a throat infection or 3–6 weeks following a skin infection (scarlatina).
• In extreme cases, may develop encephalopathy or heart failure from volume overload, HTN, and toxic effects of bacteria attacking the CNS.
• Acute phase can last 6 weeks, hematuria may persist for years.
• Diagnosis made by history, presentation, and labs.
• Documentation of a positive strep infection is helpful, or strep titer can confirm a recent infection. Anti-streptolysin O titer is elevated following a throat infection, but not a skin infection. Check deoxyribonuclease B anti-streptococcal to confirm if following a skin infection.
• Treatment is aimed at controlling effects of renal failure and HTN. Sodium restriction. ACEI, CCB, and vasodilators have been used effectively. Treat infection with appropriate antibiotic.
• Complete recovery occurs in most cases within two months. Poor recovery and poor outcomes occur when the effects of renal failure are not quickly addressed. Recurrences are rare.
Term
Glomerulonephritis Membranous glomerulopathy
Definition
• Typically presents as isolated disease, but can be secondary to autoimmune disease, malignancies, syphilis, or Hep B infections.
• Thickening of basement membrane without proliferative changes, thought to be from visceral epithelial cell deposits. Believed to be immune mediated.
• Diagnosis is made by biopsy, usually done in cases of persisting hematuria and proteinuria and lack of other explanation.
• Prognosis is variable. Most have spontaneous recovery. Up to 20% of patients develop end-stage disease. Salt restriction and diuresis can be helpful. Steroids may be of benefit in severe or prolonged cases.
Term
Glomerulonephritis Hemolytic-Uremic Syndrome (HUS)
Definition
• Most common cause of acute renal failure in kids, characterized by hemolytic anemia, uremia (toxic levels of nitrogen in the blood), and thrombocytopenia.
• Acute GI illness (E.coli) precedes 80% of cases. Can also be associated with other bacterial infections, viral illnesses, oral contraceptive use, and cyclosporin.
• Glomerular changes seen include thickening of capillary walls and narrowing of capillary lumens.
• Syndrome starts due to epithelial cell injury, leading to clotting. Anemia results as RBCs pass thru narrowed vasculature and are damaged. Platelet drop is due to damage and possible adhesion.
• Seen usually in kids under age 4. Initial illness usually includes fever, abdominal pain, bloody diarrhea, vomiting, less often a URI. 5–10 days afterwards there is a sudden onset of weakness, lethargy, pallor, irritability, and oliguria.
• PE may reveal dehydration, edema, petechiae, or hepatosplenomegaly.
• Diagnosis supported by clinical findings, history, CBC, and renal panel.
• On CBC, may see fragmented RBC, and HGB low in range of 5–9. May see helmet cells and burr cells. Platelets may be as low as 20,000.
• UA usually not remarkable. PT and PTT normal. Renal panel indicates acute failure (hyperkalemia, elevated BUN, elevated creatinine). Biopsy usually not needed.
• Treatment is supportive, with attention on HTN, nutrition, fluid, and electrolyte balance. Early dialysis decreases mortality. Antithrombotic agents not useful.
• Prognosis is good when aggressive therapy is initiated. End-stage disease occurs in about 10% of cases. Close follow-up is advised since these patients may develop HTN, proteinuria, and chronic renal insufficiency over the years.
Term
IgA Nephropathy (Berger)
Definition
• Mostcommonglomerulardisease–worldwide
• OftenpresentsfollowingURIorGIillness. Unclear pathogenesis, but known immune complex disease
• Presentswithgrosshematuria,HTN.Diagnosis made by biopsy, revealing IgA deposits
• 50%progresstoESRDinadulthood
• Supportivecare,ACEI,ARBSforHTN
Term
Alport Syndrome
Definition
• Hereditary condition, 1:50,000
• Triad: kidney, eyes, ears
• Presents with hematuria and proteinuria
• Hearing deficits: bilateral sensorineural (boys>girls)
• Ocular abnormalities: extrusion of lens into anterior chamber. Usually does not lead to complete vision loss, but central vision changes
• Diagnosis made by biopsy, genetic testing (x-linked, autosomal recessive or autosomal dominant)
• Leads to ESRD, often by age 30
• Supportive care, ACEI/ARB, dialysis, transplant
Term
Pediatric Hypertension
Definition
• Essential HTN is rare in children; it is usually secondary to underlying disease.
• Routine BP screenings should be done on all children starting at age 3. It is imperative that a pediatric cuff is used, and it should be rechecked on several occasions if only minimally elevated.
• HTN is defined as average systolic and/or diastolic reading greater than 90th percentile for age, gender, height, and weight.
• High normal BP is between 90–95th percentile.
• Significant BP is >95th percentile.
• Severe BP >99th percentile.
Term
Pediatric Hypertension
Evaluation
Definition
• May present with HA, blurry vision, UTI, edema, rash, DOE
• Family history of renal disorders
• Labs: UA, urine culture, BMP, CBC
• Imaging: renal ultrasound, EKG and may need renal angiogram in time
Term
Pediatric Hypertension
Treatment
Definition
• Non-pharmacologic: Exercise, salt restriction, weight loss. (Start here if high normal BP). Very close follow- up.
• Pharmacologic: CCB, diuretics, ACEI, B-Blockers. (The class of medication will depend largely on the underlying cause of HTN.)
• These kids should be followed by pediatric cardiology and/or nephrology.
• If hypertensive emergency, send to ED for urgent evaluation and initiation of IV antihypertensives.
Term
Dehydration
Definition
• Prolonged capillary refill
• Abnormal skin turgor
• Abnormal respirations/pulse
• Sunken fontanelles
• BUN/creatinine not very specific for infants. However, in severe dehydration you will see marked hyponatremia and hyperkalemia
Term
dehydration mild vs moderate vs severe
Definition
[image]
Term
Vesicoureteral Reflex
Definition
• It is defined as retrograde flow of urine from bladder to
ureter and renal pelvis (grades I–V).
• Ureter and bladder are normally attached, and there is a
flap-valve that prevents backflow.
• This condition occurs when the tunnel between the bladder mucosa and the detrusor muscle is short or absent, causing the flap-valve to malfunction. May resolve on own as child grows.
• Usually congenital, familial, and affects 1% of children.
• This condition causes urine to “back up,” leading to infection, inflammation, scarring. This is the leading cause of HTN in children.
• Usually found during the work-up of UTI or other renal concern. 80% of kids with reflux are female. It is typically diagnosed under age 5.
• Diagnosed by VCUG. Catheterization is required, so counseling of parent and child is important since this can be scary. Sedation is often used.
Term
Vesicoureteral Reflux
• Treatment
Definition
goal is to prevent pyelo, renal scarring, and progression to end-stage renal disease.
• Antibiotic prophylaxis is the cornerstone of treatment.
- Drugs of choice are sulfamethoxazole-trimethoprim or nitrofurantoin. These are prescribed daily at 1⁄4 the dose needed to treat an acute infection.
- Considered successful treatment if child remains UTI free and reflux spontaneously resolves as child grows.
• Toileting habits should be monitored, proper wiping techniques for girls, watch for straining with stools, and treat constipation if necessary.
• Surgical therapy may be needed if recurring UTIs or signs of renal damage. Numerous surgical techniques have been described, but in general the goal is to modify the attachment of the ureter to
the bladder.
Term
Voiding Dysfunction
Definition
• Toilettrainingbeginsatage2–3,andmostkidsare dry thru the night by age 5.
• Age5iswhencontrolofmicturitionisexpected, although it may be later in some kids.
• Nocturnalenuresisoccursmorecommonlyinboys, and there is a known family history in 50% of cases.
• Pathogenesisincludesdelayedmaturitylevelthat allow voluntary control of micturation, sleep disorders, reduced antidiuretic hormone, genetic factors, psychological factors, UTI, or other illness.
• Obtain history with attention of family history, diet history, nighttime fluid intake.
• PE involves abdominal exam, genital exam, rectal exam, neurological and spinal exam.
• If PE, urinalysis and urine culture are negative, further work-up is not indicated
Term
Voiding Dysfunction
Treatment
Definition
- Reassuranceiskey.Avoidpunitivemeasures. Limit evening fluid intake. Refer to ENT if apnea secondary to enlarged adenoids is suspected. Evaluate for psychological stressors. This can be really hard for kids since they are in the age group in which they want to have sleep-overs, visit friends, etc. Pull-ups can be helpful, and offer self-assurance. Alarms that go off when wet are available and can be helpful.
- Avoidpharmacologicaltherapyuntilage7.
• Desmopressin acetate, synthetic analog of antidiuretic hormone, reduces urine production. May cause hyponatremia, rare. No long-term adverse events reported. Taken at nighttime, dosage 0.2–0.6mg, use lowest effective dose. Use for 4–6 months, then try to taper off. If recurrence, restart at effective dose.
• Imipramine, a tricyclic antidepressant. Has anticholinergic effects. Dose is 25–75 mg at nighttime, age dependent. May cause agitation, dry mouth. Advise parents to keep drug well guarded, since it can be fatal in overdose.
Term
Voiding Dysfunction
Common causes of
Definition
daytime incontinence (not due to neurologic problem) include immaturity, bladder spasm, trauma, and genital abnormalities.
• Encouragefrequenttoiletinginkids,establish routines. Take a break during extended play.
• Labialadhesions(urethralmeatusandvagina) are common in little girls. Short-term application of topical estrogens or lysis correct this condition.
Term
Hypospadias
Definition
urethral opening located on ventral surface of penis. Due to incomplete development of the dorsal hood.
• Occurs in 1 in 250 males. Unclear etiology, may be due to in utero estrogen exposure.
• Usually an isolated finding, but can be found along with other congenital abnormalities.
• Classified by position of urethral opening and whether chordee is present (ventral curvature during erection).
• Complications include voiding dysfunction, sexual dysfunction, meatal stenosis (rare) and infertility (rare).
• Treatment: circumcision should be avoided because the foreskin may be used in surgical repair. Surgical repair is advised between 6–12 months of age. These children should be evaluated by a pediatric urologist.
Term
Phimosis
Definition
• Inability to retract the foreskin.
• In 90% of uncircumcised males, the foreskin should be retractable by age 3.
• Accumulation of epithelial debris collects under the foreskin, and hygiene is critical.
• If foreskin is not retractable, application of topical steroid TID for 3 weeks may help to loosen the skin. Circumcision is definitive treatment.
Term
Paraphimosis
Definition
• Occurs when foreskin is retracted beyond the glans penis and cannot be pulled forward again.
• Leads to strangulation of glans penis due to venous congestion.
• Lubrication may help you to push the glans penis back thru the phimotic ring and relieve the pressure, but surgical repair is often needed. This is a medical emergency.
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